Gorlin-Goltz syndrome, also known as basal cell nevus syndrome, is an uncommon autosomal dominant hereditary disorder; which is characterized by numerous basal cell carcinomas, maxillary keratocysts and bones malformations. It results from a mutation of the PATCHED gene. The estimate incidence for a general population is 1/50000 to 1/150000. We present a 30 year-old woman, with no similar famil...

Basal cell nevus syndrome (BCNS, OMIM 109400) is a familial cancer characterized by the development of numerous basal cancers and various other developmental abnormalities, including epidermal cysts skin, calcified dural folds, keratocysts jaw, palmar plantar pits, ovarian fibromas, medulloblastomas, lymphomesenteric cysts, fetal rhabdomyomas. BCNS shows autosomal dominant inheritance caused mu...