AbstractMetastatic basal cell carcinomas (mBCCs) are exceedingly rare, with an estimated variable prevalence rate between 0.003 and 0.55% among all basal cell carcinomas. Although there are hundreds of reported cases of mBCCs, a dearth of evidence exists of mBCCs in patients with Gorlin syndrome—an autosomal dominant genetic disorder. The purpose of this article was to present the first and largest case series of patients with mBCC in Gorlin. A literature review (LR) compares our data with the existing evidence base to underscore the importance of early surveillance, diagnosis and surgical intervention of suspicious lesions as distant metastases significantly reduce survival rates.