A 50-year-old man was transferred to our hospital from a local clinic due to fever and weight loss. The patient had been healthy until 15 days prior to his admission, when, at that time, he began to feel febrile, lost his sense of taste, and developed a poor appetite. The symptoms continued until the admission. Upon examination at the originating clinic, adrenal masses had been found on CT. On admission the patient's temperature was 37.7 C, pulse 80 beats/min, respiration rate 20 breaths/min, and blood pressure 120/80 mmHg. On examination, the patient appeared ill and prostrated. Liver was palpable in 1 finger-breadth on the epigastric area and a 1.5x1.0 cm sized firm and nontender right supraclavicular lymph node was palpated, but otherwise unremarkable. His leukocyte count was 1,700/muL with 65% neutrophils, hemoglobin 10.6 g/dL, and platelet 142,000/L. His blood chemistry revealed total serum protein was 6.3 g/dL, albumin 3.6 g/dL, aspartate transaminase 52 IU/L, alanine transaminase 29 IU/L, alkaline phosphatase 183 IU/L, sodium 129 mmol/L, potassium 4.0 mmol/L, lactate dehydrogenase (LDH) 1415 IU/L, and serum ferritin >2000 ng/mL. The renal function was normal. Cortisol in the morning and evening was 17.8/20 mug/dL (normal range: 5 - 25 mug/dL) and ACTH in the morning and evening 726/265 pg/dL (normal range: 10-56.7 pg/dL). Serologic test for Epstein-Barr virus and cytomegalovirus were normal. Chest radiographs obtained on admission revealed normal findings, but a CT scan of the abdomen revealed bilateral adrenal masses and hepatosplenomegaly (Figure 1). Right adrenal mass measured 6x1.7 cm and the left was 8x3 cm in size. CT-guided adrenal gland biopsy was done and showed numerous epithelioid cells and caseous necrosis consistent with tuberculosis (Figure 2). But, an AFB stain of the tissue and polymerase chain reaction for Mycobacterium tuberculosis were negative. On subsequent bone marrow aspiration and biopsy, hemophagocytic histiocytes had increased in proportion (4.6%) without accompanying evidence of malignancy (Figure 3), hence HPS associated with adrenal gland tuberculosis was diagnosed. Biopsy of the supraclavicular lymph node revealed necrosis, but no evidence of malignancy. Treatment with isoniazid, rifampin, ethambutol, and pyrazinamide was started immediately. On treatment day 4, the patient developed jaundice and elevated levels of transaminases. We discontinued hepatotoxic drugs (isoniazid, rifampin, pyrazinamide, and acetaminophen) and changed his medications to ethambutol, cycloserine, streptomycin, and ciprofloxacin. During the anti-tuberculous treatment the patient showed recurrent hypoglycemia and hypotension. Rapid ACTH stimulation test revealed adrenal insufficiency. Corticosteroid treatment was added. After supplementation with corticosteroid, the hypotension and hypoglycemia were corrected. After normalization of liver function, isoniazid was restarted with a gradual increase in dose. But pancytopenia progressed during the anti-tuberculous treatment. On treatment day 30, repeated bone marrow aspiration was performed and showed that numerous hemophagocytic histiocytes persisted (5.0%). On treatment day 41, the patient expired due to multiple organ failure (including disseminated intravascular coagulation, hepatic failure, and renal failure).