(c) Histopathology: Shows an infundibulocystic basal cell carcinoma characterized by a relatively well-circumscribed neoplasm showing cystic areas as well as a cleft in the adjacent dermis (hematoxylin-eosin stain original magnification ×10) gl The patient's mother was a 77-year-old woman with a medical history of a single facial nodular BCC, a frontobasal meningioma, and a mandibular plexiform ameloblastoma. Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome (GS), is a tumor predisposing disorder with an autosomal dominant inheritance.1,2 It is caused by mutations in the hedgehog pathway, being I PTCH1 i the most commonly affected gene. Many other syndromes are also associated with the development of multiple facial papules and solid organ neoplasias and should be considered in the differential diagnosis (Table 2).2,10 Patients with I SUFU i mutations also seem to develop less odontogenic keratocysts when compared with patients harboring I PATCHED i mutations. [Extracted from the article]