Tumors of the anus and perianal skin are rare. Their presentation can vary and often mimics common benign anal pathology, thereby delaying diagnosis and appropriate and timely treatment. The anatomy of this region is complex because it represents the progressive transition from the digestive system to the skin with many different co-existing types of cells and tissues. Squamous cell carcinoma of the anal canal is the most frequent tumor found in the anal and perianal region. Less-frequent lesions include Bowen's and Paget's disease, basal cell carcinoma, melanoma, and adenocarcinoma. This article aims to review the clinical presentation, diagnostic evaluation, and treatment options for neoplasms of the anal canal and perianal skin.