[Basal cell nevus syndrome with squamous cell carcinoma of the larynx]

We report on a 54-year-old patient suffering from basal cell nevus syndrome. At the age of 51, squamous cell carcinoma of the larynx had been diagnosed. The frequent occurrence of various kinds of benign and malignant neoplasms in patients with basal cell nevus syndrome illustrates the close relationship between this syndrome and phacomatoses.