Basal cell carcinoma is the most common malignant tumor in the fair-skinned population and its incidence continues to rise. An update of the S2k guideline with the participation of all specialist societies familiar with the clinical picture and previous literature research is of great importance for the quality of care for affected patients. In addition to epidemiology, diagnostics and histology are discussed. After risk stratification, therapy is divided into topical, systemic and radiation therapy. Surgical removal remains the treatment of first choice in most cases. The approval of anti-PD1 inhibitors for locally advanced and metastatic tumors has opened up a new option in second-line therapy (after hedgehog inhibitors).