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Differential diagnosis: According to Haywards criteria, diagnosis of a primary melanoma must consider the following factors: 1) malignant melanoma outside the CNS was not detected, 2) absence of this lesion in other sites in the CNS, and 3) the intramedullary lesion was confirmed pathologically ( 4.1 Meningeal melanocytoma: According to Brat, meningeal melanocytoma and malignant melanoma both arising from the normal melanocytic cells in the leptomeninges are the two extremes of a spectrum of primary melanocytic neoplasms ranging from low-grade to high-grade in the CNS ( 4.2 Metastatic malignant melanoma: Although the CNS is a common site of metastases from malignant melanoma, which is the third most common neoplasm to metastasize to the CNS, spinal metastatic melanoma is extremely rare with accompanying multiple lesions in other sites of the CNS ( 4.3 Other melanotic tumors 4.3.1 Neurocutaneous melanosis: Neurocutaneous melanosis is one of the most infrequent neoplastic lesions of the CNS, which is characterized by the presence of congenital melanocytic cutaneous nevi associated with intracranial leptomeningeal melanocytosis

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