Neurofibromatosis (NF) is a disorder with a wide spectrum of clinical manifestations. Here, we describe a 16-year-old boy with NF1 who had pseudoarthrosis of the 4th and 5th fingers of the left hand. He had specific cutaneous lesions and Lisch nodules in the iris. Because NF1 affects multiple organ systems, patients are likely to benefit most from a multidisciplinary treatment strategy.