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Christian Medical College, Department of Neurosurgery, Ludhiana, India DOI : 10.5137/1019-5149.JTN.5272-11.0 Melanocytomas are rare melanocytic tumors of the central nervous system and its presence at the foramen magnum is extremely rare. We report a case of a 55-year-old male presenting with progressive quadriparesis over one year. Imaging showed a well-defined intradural extramedullary lobulated mass at craniovertebral junction towards the left side and extending to left C2-3 neural foramina. Patient was operated through foramen magnum approach with near total excision of tumor. On a ten-month follow up, he was ambulatory with normal motor power on right side of body and left lower limb and with motor power of 4-/5 in left upper limb. Histopathology and immunohistochemistry confirmed the lesion to be a melanocytoma. Keywords : Melanocytoma, Foramen magnum, Craniovertebral junction