A case of malignant melanoma discovered at the base of the skull is reported in a 52-year-old male. The patient with no previous significant history, complained of headaches. He developed progressive paralysis of the IX, X and XI left cranial nerves and a Claude-Bernard-Horner syndrome. The tumor, discovered at the nervous compartment of the jugular foramen was treated by surgery and radiotherapy. The patient died 27 months after surgery. The absence of other systemic localisations allows to consider this melanoma as primitive. The presence of spindle cell areas in the tumor may suggest the diagnosis of melanotic schwannoma. Immunohistochemistry is still disappointing because of the lack of specific markers. Our results, in agreement with those of the literature, emphasize the importance of the histopathological findings and the determining role of the electron microscopy in the diagnosis and the differential diagnosis of these two entities, whose nosological frontiers may, sometimes, be difficult to distinguish.