We present the rare case of solitary xanthogranuloma in the upper cervical column mimicking a Brown-Sequard syndrome. A 29-year-old man complained with right hemiparesis and left hypoesthesia after a car accident. Computed tomography and magnetic resonance images revealed a lobulated homogenously well-enhancing mass in between posterior arch of the atlas (C1) and spinous process of the axis (C2) resulting in a marked spinal canal narrowing with cortical erosions. The patient was managed by complete resection of the tumor with partial laminectomy with lower half of C1 posterior arch and upper half of C2 spinous process. The authors advise complete removal of the xanthogranuloma and consideration as a differential diagnosis of lesions among upper cervical lesions.