Neurofibroma type I (NF1) is an autosomal dominant disorder of the nervous system caused by mutations in the NF1 gene, with a global incidence of about 1/3 000. Neurofibroma is a typical clinical manifestation of NF1, and can be divided into nodular, plexous and diffuse neurofibroma according to their pathological characteristics, among which diffuse neurofibroma is the most rare. We report a rare case of NF1 with diffuse external nasal neurofibroma with laryngeal hyperplasia. The clinical data are as follows.