Basal cell carcinoma is a slow-growing, malignant epidermal tumor predominantly affecting sun exposed areas in Caucasians, accounting for up to 80% of all diagnosed skin cancers, with a rising incidence. Chronic UV radiation, in association with constitutional factors, plays the main role in its etiology. Inappropriate activation of the hedgehog signaling pathway seems to be a key pathogenesis mechanism. Basal cell carcinoma metastases are extremely rare, but it is a locally invasive tumor that can cause significant destruction of the surrounding tissues, with their functional and esthetic impairment. There are four main clinical types of basal cell carcinoma, although clinical classification is of poor prognostic significance. Preselection of suspicious lesions and treatment planning include noninvasive diagnostic techniques: dermoscopy, confocal microscopy and ultrasoud imaging, yet histopathology remains the “gold standard” of basal cell carcinoma diagnosis. In terms of the histological growth pattern, which is essential for the prognosis, basal cell carcinoma may be divided into circumscribed or diffuse types. Surgical excision is considered to be a first line treatment option, but there are numerous less invasive treatment modalities for low-risk basal cell carcinoma. Prevention strategies are focused on behavioral modifications, regular follow up and use of chemopreventive agents in high-risk patients.