Objective: To investigate the clinicopathological features of melanocytoma of central nervous system. Methods: The clinical manifestations, histopathological and immunohistochemical results were analyzed in one case of melanocytoma occurring in the spinal cord with reviewing the related literatures. Results: The patient was a 52-year-old woman presenting a not flexible right limb for 9 months. MRI showed that an abnormal round well-circumscribed mass was observed on vertebral 3-4 level, measured 3.7 cm × 0.8 cm × 1.3 cm. Microscopically, the tumor consisted of round, oval or spindle cells, formed tight nests with a superficial resemblance, arranged in bunchiness or whorls. Heavily pigmented tumor cells and tumoral macrophages were seen at the periphery of nests. Melanocytoma cells showed oval or spindled nuclei with small nucleoli and clear to eosinophilic cytoplasm with variable fine pigment. Cytologic atypia and mitoses were absent. Immunohistochemical study showed that Vimentin, HMB45, S-100, Melan-A were positive, Ki67 proliferation index was 5%~10% , other antibodies including Ckpan, EMA, GFAP, CD34, SMA, Desmin and P53 were all negative. Conclusion: Melanocytoma is a rare tumor of intermediate malignant potential, which is difficult to be distinguished from diffuse melanocytosis and melanomatosis, malignant melanoma, and other tumors with melanin differentiation. Definitive diagnosis requires histological examination, supplemented with immunohistochemistry, radiological characterization and ultrastructural findings. The complete resection of the lump with post-operative follow-up, as well as local radiotherapy, is the main modality of treatment.