Basal cell carcinoma (BCC) is the most common cancer of the Caucasian race and arises in the skin. The primary risk factor for BCC is ultraviolet radiation, which explains why most tumours are located to sun-exposed areas, such as the face. BCC typically grows slowly over years, and 10-year survival of patients is actually higher than that of the general population. However, BCC can rarely be locally advanced with local, occasionally extensive, tissue destruction or even metastasis. It typically first appears as a small, inconspicuous, skin-coloured, pearly papule that slowly progresses into an ulcerated nodule (nodular subtype, “rodent ulcer”). Localized BCCs are categorized into low and high risk based on a set of clinical and histopathological criteria, and this guides therapeutic decisions (the TNM system is not used). The treatment recommended for low-risk primary tumours is standard surgical excision. Mohs micrographic surgery is superior to standard excision for tumours of the face (primary or recurrent) in terms of recurrences. It should be considered for primary high-risk BCCs and is recommended as first-line treatment for recurrent BCCs with at least one more high-risk feature, especially high-risk location. Radiotherapy should be considered for (preferably) adults ≥60 years of age with low- or high-risk BCCs, in case MMS and standard excision are unsuitable. Vismodegib per os, a Hedgehog-signalling pathway inhibitor, is the first-line treatment for metastatic BCC, and cemiplimab, a monoclonal antibody, is recommended for locally advanced or metastatic BCCs, in case vismodegib has failed.