Anal cancer accounts for only 1.5% of gastrointestinal malignancies but this disease has shown a steady increase in incidence particularly in HIV positive males. The understanding of pathophysiology and treatment of anal cancer has changed radically over last thirty years. Risk factors have been identified and organ preservation by chemoradiotherapy has become a standard. This article aims to review the clinical presentation, diagnostic evaluation, and treatment options for anal cancer in the light of current literature.