The majority of colorectal and anal malignancies are adenocarcinomas and squamous cell cancers, respectively. Despite the predominance of these neoplasms in these locations, rare histiotypes of the colon, rectum, and anus do occur. These histotypes include but are not limited to lymphoma, melanoma, diffuse cavernous hemangioma, and sarcomas, such as leiomyosarcoma or Kaposi's sarcoma. These tumors often present challenges to clinicians with respect to diagnosis, staging, management, and pathology because of their unfamiliarity. A Medline search using "colon," "rectum,""anus," "lymphoma," "melanoma," "diffuse cavernous hemangioma," "squamous cell carcinoma," "carcinoid," "sarcoma," "leiomyosarcoma," "Kaposi's sarcoma," "Paget's disease," "Bowen's disease," and "basal cell carcinoma" as key words was performed as well as a cross-referencing of the bibliography cited in each work. Rare tumors of the colon, rectum, and anus present diagnostic and management dilemmas for clinicians. Because of their infrequency and poor prognosis, the optimal management of these tumors is controversial. For some histotypes, such as squamous cell carcinoma and carcinoids of the rectum, treatment depends on location and size of the tumor. For uncommon anal lesions, such as Bowen's disease, Paget's disease, and basal cell carcinoma, wide local excision (WLE) with negative margins is the standard of care. For other lesions such as anorectal melanoma or leiomyosarcoma, abdominal perineal resection versus WLE is still being debated. Because the optimal treatment of these tumors is still unclear, we recommend a multidisciplinary approach including a surgeon, primary care physician, medical oncologist, radiation oncologist, and pathologist to offer the patient the best outcome.