The gastrointestinal (GI) tract is a common internal organ to be involved by human immunodeficiency virus (HIV)-related malignancies. It is the second most common site for Kaposi sarcoma after skin, and the commonest visceral site, for Kaposi sarcoma in AIDS patients. GI lymphomas have been documented in approximately 25% of AIDS patients with systemic lymphomas. Moreover, GI involvement of AIDS-lymphoma has been associated with poor prognosis and short survival. Several other malignancies that occur in the GI tract are also closely related to HIV-infected or immunosuppressed individuals; these include posttransplant lymphoproliferative disorder, Epstein-Barr virus-associated smooth muscle tumors, anal precancerous lesions, and squamous cell carcinoma. As a result of active antiretroviral therapy, patients infected with HIV are living longer and are consequently at increased risk for development of cancer. Therefore, it is possible that the number of AIDS-associated malignancies will rise and the pattern of tumors may change in the future. In this paper, the clinicopathologic features of GI malignancies associated with AIDS patients are reviewed and the differential diagnosis with other mimic lesions is discussed.