BACKGROUND: Basal cell carcinoma (BCC) is extremely common, and its incidence continues to rise. OBJECTIVE: This review presents the literature pertaining to the epidemiology, pathogenesis, and risk factors associated with BCC. CONCLUSIONS: The risk of developing BCC depends on both genetic predisposition and exposure to risk. Fair-skinned people account for the overwhelming majority of patients, beyond what would be expected by skin phototype alone. Damage to multiple lines of defense appears to be necessary for cancer development and spread. This damage distorts the concerted effort of deoxyribonucleic acid (DNA) repair, immunosurveillance, and cellular growth regulation to protect against malignant progression. Ultraviolet light exposure is the most critical modifiable factor determining early expression and frequency of BCC development.