A 66-year-old Japanese woman presented with bilateral blurred vision 4 months prior to visiting our hospital. She had been diagnosed with conjunctivitis and prescribed antibacterial eye drops by a local ophthalmology clinic. While undergoing therapy, she developed general fatigue and lumbar pain. She visited a private clinic, and was diagnosed with bilateral exudative pleural effusions. She was then referred to our hospital for further investigation and treatment. After admission to our hospital, complete physical examinations were done. Her exudative pleural effusion was of unknown origin. The patient had a past history of breast cancer 2 years ago, but no malignant cells were observed in cytology examination, and no malignancy was detected by CT and MRI. In pleural fluid examination, the specific gravity was 1.304, cell count 397/microL (neutrophils 4.0%, lymphocyte 62.0%, macrophage 34.0%), lactate dehydrogenase 105 U/L, adenosine deaminase 8.7 U/L, total protein 4.4 g/dL, rheumatoid factor < 10.0 IU/mL, antinuclear antibodies (+-), and negative cultures. The cause of lumbar pain was also examined by the orthopedic department, and she was diagnosed with multiple spondylitis. Findings of an MRI of the spine and bone scintigraphy are shown in Figure 1. She also had palmoplantar pustulosis beginning several years ago. Based on the findings of spondylitis and palmoplantar pustulosis, she was diagnosed with SAPHO syndrome by both orthopedists and dermatologists, and was prescribed antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs). Seven days after admission, she was referred to ophthalmology for investigation of the cause of her blurred vision. At presentation, her best-corrected visual acuity (BCVA) was 0.6 in both eyes. Intraocular pressure was 18 mm Hg in the right eye and 16 mm Hg in the left eye. Slit-lamp examination demonstrated bilateral shallow anterior chambers. There were no keratic precipitates in both corneas, and no cells in the anterior chambers. Fundus examination revealed bilateral annular choroidal detachment with exudative serous retinal detachment involving the macula. Optical coherence tomography (OCT) showed subretinal fluid with a wavy retinal pigment epithelium (RPE) line and thicker choroid in both eyes. Fluorescein angiography (FA) depicted multiple areas of pinpoint dye leakage from the RPE. Indocyanine green angiography (ICGA) showed multiple areas of choroidal hypoperfusion in both eyes (Fig. 2). B-scan ultrasonography confirmed choroidal detachment with no associated scleral thickening or masses affecting the choroid and sclera. Although the patient did not meet all the diagnostic criteria for VKH disease, differential diagnosis showed no evidence of diseases such as uveal effusion syndrome, posterior scleritis, or choroidal metastasis, and laboratory markers of other types of uveitis including sarcoidosis, syphilis, and tuberculosis were negative. Moreover, autoimmune diseases such as rheumatoid arthritis and antineutrophil cytoplasmic antibody-associated vasculitis, which are often accompanied by uveitis, were also unlikely because of the absence of antibodies specific to these diseases. Her clinical findings including FA, ICGA, and OCT indicated VKH disease. Treatment was begun with a subtenon injection of triamcinolone acetonide in both eyes, while internists continued investigations to rule out other diseases. After 7 days, when internists finished the diagnostic workup with no additional findings, high-dose intravenous corticosteroids were given for 3 days. Treatment was continued with a slowly tapering course of oral prednisolone to 30 mg per day for 8 days. Because the choroidal and retinal detachments persisted in spite of steroid therapy, the patient was prescribed immunosuppressive medication of cyclosporine 150 mg twice daily. Two weeks after starting cyclosporine, cellulitis occurred in the left leg and C-reactive protein (CRP) increased to 23.5 mg/dL. Cyclosporine was discontinued, and prednisolone was further tapered to 10 mg per day. Because of this episode, ocular findings persisted and worsened. Ten days after discontinuing cyclosporine, her general condition improved and CRP level decreased to within the normal limit. Treatment was resumed with high dose (1 g) of intravenous corticosteroids for 3 days and cyclosporine 150 mg twice daily. Seventy days after the second course of high-dose intravenous corticosteroids, choroidal and retinal detachments were resolved completely, and BCVA was 0.5 in the right eye and 0.7 in the left eye (Fig. 3). Bilateral pleural effusions were also resorbed, and her general condition improved.