Basal cell carcinoma (basalioma, BCC) is the most common skin cancer and the most common human malignancy in general, with a continuously increasing incidence. In most cases, BCC develops on chronically sun-exposed skin in elderly people, most commonly in the head and neck region. Besides chronic UV radiation, other risk factors for the development of BCC include sun bed use, family history of skin cancer, skin type 1 and 2, a tendency to freckle in childhood, immunosuppression, previous radiotherapy, and chronic exposure to certain toxic substances such as inorganic arsenic. There are numerous variations in clinical presentation of BCC, such as nodular BCC, ulcerating BCC, pigmented BCC, sclerosing BCC, superficial BCC, and fibroepithelioma of Pinkus. Each varies in terms of clinical presentation, histopathology and aggressive behavior. Treatment modalities for BCC include surgical excision, cryosurgery, curettage, electrodessication, radiotherapy, photodynamic therapy, topical cytostatics, and immunomodulators. If left untreated or inadequately treated, BCC may become invasive and locally destructive, although it very rarely metastasizes. Due to the extremely high incidence of BCC, medical professionals should be familiar with its manifold clinical presentations.