Multiple endocrine neoplasia type 1 (MEN-1) is an autosomal dominant syndrome consisting of endocrine tumors of the parathyroid gland, pituitary gland, and pancreas. MEN-1 is caused by loss of function mutations in the MEN1 gene, which encodes the tumor suppressor protein menin. Cutaneous collagenomas and facial angiofibromas also have been associated with MEN-1 and may serve as diagnostic clues to the diagnosis. We present a case of amelanotic melanoma resembling a large angiofibroma in a young man with MEN-1.