Congenital "cafe au lait" spots are present in more than 90% of patients with neurofibromatosis. This autosomal dominant disorder is characterized by multiple neural tumors, especially neurofibromas, pigmented hamaomas of the iris (Lisch nodules), and cafe au lait spots, which usually occur over nerve trunks. Although the majority of neurofibromas in this disease are benign, the tumors can be quite disfiguring and psychologically damaging. Basal cell carcinoma is a common, slow-growing tumor of sun-exposed skin. It develops in adulthood after years of chronic sun damage. Although malignant melanoma may arise from large congenital nevi, BCC does not. Neuroblastoma is a common childhood tumor that arises anywhere along the sympathetic chain, and most commonly in the adrenal medulla. The tumor usually presents as an abdominal mass and is not associated with skin findings. The ocular neoplasm associated with neurofibromatosis is the Lisch nodule, not the retinoblastoma. Retinoblastoma is a neuroepithelial tumor usually identified by funduscopic examination of a child with visual changes. Ref: Listernick R., Charrow J. (2012). Chapter 141. The Neurofibromatoses. In L.A. Goldsmith, S.I. Katz, B.A. Gilchrest, A.S. Paller, D.J. Leffell, N.A. Dallas (Eds),Fitzpatrick's Dermatology in General Medicine, 8e.