Basal cell carcinoma (BCC) is the most common malignant tumor among human neoplasms. BCC appears in the sun-exposed areas, most often in the face, above the imaginary line that connects lip angles with ear laps. The majority of BCC cases are triggered by DNA mutations produced by UV radiation. Apart from ultraviolet radiation there are other exogenous carcinogens such as exposure to the ionizing radiation, arsenic, industrial chemical substances etc. Also one of the most significant ethiological factors appears to be a genetic predisposition. Altough majority of BCC are highly suspected clinically, in order to make final diagnose, a skin biopsy is performed for pathohistological verification. Different clinical presentations of BCC are: nodular basalioma, pigment basalioma, infiltrated basalioma, superficial basalioma, morpheiform basalioma, cystic basalioma, fibroepithelioma, metathipical basalioma and metastatic basalioma. BCC progresses slowly and altough it metastasizes rarely, it can cause significant destruction and disfigurement by invading surrounding tissues. Perivascular or perineural invasion are less common and are associated with the more agressive clinical forms. Numerous therapeutic approaches have been developed for treatment of BCC. Each of these methods has its limitations and neither of them doesn't completely prevent the risk of relapse. The aim of treatment is to remove the tumor to the margin of normal tissue with most acceptable cosmetic outcome. Surgical treatment represents standard therapy method for BCC removal. Examples of the variety of treatment modalities used are surgical excision, curretage and electrodessication, cryosurgery, radiation therapy, Mohs' micrographic surgery, laser surgery, photodynamic therapy, imiquimod, and 5-fluorouracil. Adequate treatment option is generally chosen based upon factors such as localization and size of the tumor and patient's age and comorbidity. Recent advances in understanding the biologic pathways in the development and progression of BCC, have also led to the development of the first molecular targeted therapy for this disease, vismodegib.