Lipofibromatous hamartoma of the median nerve associated with macrodactyly and port-wine stains.

Lipofibromatous hamartoma of the nerve is a very uncommon congenital tumor. An association between this condition and vascular malformations is not well known. We present an 11-year-old girl with a lipofibromatous hamartoma of the right median nerve with macrodactyly. She had small red macules on her right neck, chest, and arm, which were diagnosed clinically as port-wine stains. The specimens of the enlarged nerves showed fibrous and fatty growth surrounding the nerve bundles and proliferation of the small veins. We suggest that this disorder can be accompanied by a vascular malformation.