Melanotic schwannomas are tumors of Schwann cell origin, characterized by cytoplasmic deposition of melanin. Melanotic schwannomas are rare. Description of the course of these tumors differs somewhat, but it is generally considered a benign lesion.A 54-year-old man presented with hypoesthesia, pain and weakness of the right arm and leg for 4 months. An MRI scan revealed an intradural extramedullary lesion at the level of the foramen magnum and C1. OPERATION: Total resection of the mass was performed. A diagnosis of melanotic schwannoma was made based on histologic morphology and the immunohistochemical profile. Over a 2-year follow-up period there has been no local recurrence.Melanotic schwannoma is a rare variant of schwannoma composed of melanin-producing cells with ultrastructural features of Schwann cells. Distinguishing between this tumor and malignant melanoma is important in planning management. Total resection should be performed. Appropriate long-term follow-up is needed for all melanotic schwannomas.