A 16-year-old girl had a three-year history of many cafe au lait spots
and freckles in a dermatomal distribution. The diagnosis of segmental
neurofibromatosis (NF-5) was made on the basis of the clinical featur
es, distribution of the lesions, and absence of systemic involvement.
Only a few cases in the literature describe NF-5 with only macular les
ions. An accurate clinical examination is important to identity this u
nusual presentation.