An updated review of management of nevoid basal cell carcinoma syndrome (NBCCS) is presented. An ideal treatment of NBCCS does not exist, and surgical intervention has been the most commonly used treatment, as it provides excellent cure rates. However, patients with NBCCS typically present with a large number of basal cell carcinomas (BCCs) with repeated occurrence throughout life. Surgical intervention, although efficient, may be too painful and discomforting, especially if it has to be frequently performed. Additionally, depending on the location and extent of the tumor, the risk for cosmetic or functional defects exists. This article investigates and compares current alternative, minimally invasive treatment modalities and their potential benefits and success rates.