The nevoid basal cell carcinoma syndrome is a hereditary disorder of which basal cell carcinomas, jaw cysts, and skeletal anomalies are the major features. In two of the earliest reported cases, fibrosarcoma of the jaw was present. A failure of this lesion to have been reported in subsequent cases has raised some doubt as to whether or not it should be included in the syndrome. The present case report documents a fibrosarcoma of the jaw in association with other lesions of the syndrome and supports this relationship.