Basal cell carcinoma (BCC) is the most common nonmelanoma skin cancer. If left 
untreated, BCCs can become locally aggressive or even metastasize. Currently 
available treatments include local destruction, surgery, and radiation. Systemic 
options for advanced disease are limited. The Hedgehog (Hh) pathway is 
aberrantly activated in a majority of BCCs and in other cancers. Hh pathway 
inhibitors are targeted agents that inhibit the aberrant activation of the Hh 
pathway, with smoothened being a targeted component. Sonidegib is a novel 
smoothened inhibitor that was recently approved by the US Food and Drug 
Administration. This review focuses on BCC pathogenesis and the clinical 
efficacy of sonidegib for the treatment of advanced BCC.