A case of cervical juxtamedullary meningeal melanocytoma is presented along with results of careful neuroradiological and pathological examinations. The authors review the previous literature and discuss the problems related to the intriguing diagnosis of this very rare entity. CT and MRI show variable appearances due to different degrees of melanization and do not reliably allow us to distinguish meningeal melanocytomas from other pigmented tumors. Pathological examination of the tumor is critical for diagnosis, but it absolutely must comprise electron microscopy and immunohistochemistry. Immunohistochemical and ultrastructural criteria are thus proposed to distinguish meningeal melanocytoma from pigmented meningioma or schwannoma and malignant melanoma (primary or secondary).