Basal cell carcinoma (BCC) is the most common skin malignancy worldwide. Ultraviolet light exposure is the best known exogenous factor in BCC development. This is also the target for primary prevention. Advanced BCC include locally advanced tumors and metastatic tumors. Prognosis is worse compared to stage I and II BCCs. Mohs or micrographically controlled surgery is the gold standard of treatment. In patients with tumors that cannot be completely removed radiotherapy was the only alternative in the past. More recently new drugs for targeted therapy of signaling pathways like sonic hedgehog or epidermal growth factor receptor became available. More small molecules are under investigation. Since the complete response rates are limited, future research has to evaluate their combination with surgery.