Basal cell nevus syndrome is a rare, autosomal dominant disorder, predominantly caused by a mutation in the PTCH1 gene. As basal cell carcinomas and keratocysts are the most common abnormalities, dermatologists, orofacial maxillary surgeons, and dentists play a key role in patient care. From the age of 8, screening for odontogenic keratocysts with an orthopantomogram or MRI is recommended every other year. The intensity increases to annual screening after the development of the first odontogenic keratocyst. If BCNS is caused by an underlying SUFU mutation, screening is not indicated since there are no reports of odontogenic keratocyst in these patients to date. Radiation exposure by, for example, computed tomography, should be minimized as it induces new BCCs. Regular follow-up by a dermatologist for early diagnosis and treatment of (multiple) BCC's is recommended for life.