Neurofibromatosis (NF) is a genetically inherited, autosomal-dominant disease with an incidence of 1/3,000 in live births. There are two types of NF, NF 1 and NF 2, and NF 1 is the most common type. This study reports on the diagnosis, treatment, and related family medical history of a rare case with NF-1 in the right lower leg.