Neurofibromatosis type 1 (NF1) is a tumor predisposition disease. Multiple neurofibromas are among the characteristic tumors of NF1. The report describes the diagnosis and treatment of a large spinal neurofibroma in a NF1 patient. The tumor showed a striking pigmentation and was diagnosed as pigmented (melanotic) neurofibroma. The distinction between this rare tumor variant and other pigmented tumors, especially malignant melanoma, is of primary importance.