Objective Angiolipoma is a benign tumor that rarely occurs in the gastrointestinal (GI) tract. This case series was performed to increase clinicians’ understanding of the diagnosis and management of GI angiolipoma. Methods We evaluated the clinical presentations, diagnoses, and treatments of 3 patients with angiolipoma in the upper GI tract in our hospital and 27 cases reported in the literature. Results Among all 30 cases of GI angiolipoma, 2 originated in the esophagus, 4 in the stomach, 2 in the duodenum, 1 in the jejunum, 7 in the ileum, 1 in the cecum, 9 in the colon, 2 in the rectum, and 2 in both the duodenum and colon. Conclusion Angiolipoma of the GI tract may be more likely to occur in middle-aged and elderly men and be located in the ileum and colon. The clinical manifestations generally involve upper GI hemorrhage and lower GI pain and obstruction. Computed tomography and endoscopy may be effective diagnostic techniques, but postoperative histopathologic examination is still the gold standard. The management of angiolipoma in the GI tract depends on the size and type of lesion. Most cases in the present series were managed by complete surgical resection, after which no recurrence was found.