This 48-year-old female came with insidious-onset gradually progressive, continuous, dull aching type of pain on the right side of the neck radiating to the vertex region for 3 months. H/o weakness of all the four limbs along with tingling and numbness (Left >Right) were noted for 1 week, which was associated with bladder retention for 3 days. Motor examination showed increased tone in all four limbs. Power on the right upper limb was 4/5, power on the left upper limb was 0/5, lower limbs bilaterally was 3/5, left plantar was up going, sensory examination revealed decreased sensation over the left C2 dermatome and all modalities decreased below C2. X-ray of the cervical spine showed osteolytic destruction of the C2 body with subluxation of the C1 and C2 complex over C3 [Figure 1]. Magnetic resonance imaging (MRI) of the cervical spine showed kyphosis of the cervical spine with destruction of the C2 vertebral body along with pathological fracture. MRI also showed that the C2 vertebral body was showing T1 hypointense, T2 hyperintense lesions with posterior displacement of the severely compressed spinal cord [Figure 2]. Differential diagnosis of the expansile C2 vertebral body in immunocompetent and immunodeficiency patients is enumerated in Table 1. Preoperatively, our presumptive diagnosis was C2 vertebral body tuberculosis. The patient underwent decompression of the C2 lesion through the C2 right pedicle with occipito-C1-C3 lateral mass screws fixation (vertex medtronic system) [Figure 3]. Lesion anterior to the cord was reached by a transpedicular approach and decompression was performed. The lesion was pinkish grey, firm and moderately vascular and was destroying the C2 vertebral body. Preoperative and intraoperative opinion was tuberculoma; therefore, safe maximal resection was performed instead of complete removal. The patient improved symptomatically in power in the left upper limb and lower limb over the next 1 week duration from 0/5 to 4+/5. Histopathological examination of the tumor tissue showed spindle cells arranged in whorls and fascicles with proliferation of smooth muscle cells surrounding the blood vessels. Spindle cells showed eosinophilic cytoplasm with elongated nuclei with blunt ends. There was moderate cellularity, minimal atypia, inconspicuous mitosis and no evidence of necrosis [Figure 4a and b]. The tumor cells stained positively for smooth muscle actin, desmin and vimentin and negative for epithelial membrane antigen, S-100, Cluster of differentiation 34, estrogen and progesterone receptors, confirming the diagnosis of the leiomyoma [Figure 4c and d] and excluding the other possible lesions like meningioma and schwannoma. The patient was evaluated with ultrasound abdomen and contrast tomogram of the chest, abdomen and pelvis to rule out other possible lesions in the lung, intestines and uterus. Second surgery (trans-oral) was planned based on histopathology for complete removal of the tumor, which was postponed because of drastic improvement in myelopathy features. Postoperative MRI at 1 year showed residual tumor without cord compression [Figure 5], and she was asked for regular follow-up for any recurrence of symptoms.