Background: Kaposi sarcoma (KS) is an angioproliferative tumor caused by human herpesvirus 8 (HHV-8). Gastrointestinal (GI) involvement by KS is a rare endoscopic finding, scarcely characterized in the literature. Objective: To characterize clinical and endoscopic features of patients with GI KS. Methods: This is a single-center retrospective study of GI KS cases confirmed by immunohistochemistry in the last decade (2006-2015). The following variables were analyzed: demographic data; clinical data (extraintestinal involvement, symptoms, presence and stage of HIV infection, immunosuppressive therapy); endoscopic data; stage-stratified therapeutic approach; and mortality (at 3 and 6 months). Results: Thirteen patients with GI KS were identified: 77% were men, the mean age was 55 years, and 62% of them were Native Africans. In most cases (n = 10, 77%), KS was associated with HIV. A total of 90% of the HIV patients had a CD4+ count of <200/μL (C3, CDC classification), and 80% of them had KS as the initial manifestation of HIV infection. Thirty percent of the cases had other AIDS-defining illnesses, and only 20% received antiretroviral therapy. In the remaining 3 patients (23%), KS was associated with immunosuppressive therapy. Most patients (85%) had cutaneous lesions and 15% lung involvement. In most cases (85%), the lesions were diagnosed in the upper digestive tract in asymptomatic patients (7 stomach; 2 stomach and duodenum; 2 esophagus). Colonic involvement occurred in 2 patients presenting with hematochezia. Nearly half of the patients had more than 3 endoscopic lesions and the most frequent morphologic type was polypoid/nodular (62%). Treatment was based on antiretroviral therapy or reduction of immunosuppression and in 39% of the patients on administration of doxorubicin. Survival at 3 and 6 months was 46 and 39%, respectively. Conclusion: GI KS is mostly found in nontreated, stage 3, HIV patients, and particularly in men from areas where HHV-8 is endemic. Involvement of the upper digestive tract is often asymptomatic. The endoscopic appearance is variable and these patients have a poor prognosis.