It has been postulated that ovarian fibrothecomas are etiologically associated with a tumorigenic syndrome called nevoid basal-cell carcinoma syndrome, also known as Gorlin syndrome, or abnormalities in the candidate gene, PTCH. Gorlin syndrome is an autosomal dominant syndrome that presents with neurological and musculo-skeletal anomalies, unusual facial appearance, and a higher risk of basal cell carcinoma at or around puberty. This evidence concerns the gene PTCH1 and nevoid basal cell carcinoma syndrome.