CONTEXT: The anal canal possesses complex anatomy and histology and gives rise to a variety of tumor types. Challenging issues remain with regard to both the pathologic diagnosis and the clinical management of these tumors. OBJECTIVES: To provide an updated overview of the histogenesis, clinical and pathologic characteristics, diagnostic terminology, and relevant clinical management of the various types of anal canal tumors. DATA SOURCES: Recent literature on clinical and pathologic characteristics of anal canal tumors. CONCLUSIONS: Although most anal canal tumors are of squamous lineage, a complex variety of other tumors also occurs. Recognition of such diverse tumor entities will allow accurate pathologic diagnosis and most optimal clinical management.