This report concerns a 66-year-old man with a melanocytoma arising at the foramen magnum. Magnetic resonance imaging disclosed a well-circumscribed tumor extending from the medulla oblongata to C1 with gadolinium enhancement. A heavily pigmented tumor located under the leptomeninges was removed surgically. Although the patient died 8 months later of renal cell carcinoma, no recurrence or metastasis of the melanocytoma was detected by radiographic examination. Microscopically, the resected tumor was composed of polygonal to spindle-shaped cells containing large amounts of melanin. The bland nuclei of the tumor cells were of uniform size. No mitotic figures were seen. The tumor cells were positively immunostained for S-100 protein and by antibody HMB-45. They were not stained using the Ki-67 (MIB-1) antibody, indicating low proliferative activity. The ultrastructural examination revealed numerous mature melanosomes and basal laminae surrounding nests of cells. The tumor was diagnosed as a melanocytoma on the bases of its microscopic features and the lack of Ki-67 immunoreactivity. The ultrastructural and immunohistochemical features of melanocytomas are distinct from those of meningiomas. It is likely that melanocytomas and melanotic schwannomas represent opposite extremes of the continuous spectrum of neuroectodermal tumors derived from the neural crest.