Rationale:Splenosis is the heterotopic auto-transplantation of the splenic tissues. Gastric splenosis in a rare location mimics a gastrointestinal stromal tumor (GIST). Gastric splenosis with hemangioma has not been reported throughout the literature.Patient concerns:We report a case of a 74-year-old schistosomiasis cirrhosis splenectomy woman diagnosed with gastric fundus mass. Preoperative computed tomography and endoscopic ultrasonography revealed findings suggestive of a GIST.Diagnoses:The mass located in the gastric fundus muscularis propria, measuring 3.9 × 2.8 × 2.4 cm with a dark red color, was removed by surgery. In the mass, a 1 × 1-cm red-purple nodule was also found. On microscopic examination, a well-formed splenic tissue divided into two compartments–white pulp and red pulp–separated by an ill-defined interphase known as the marginal zone. However, a nodule in the heterotopic spleen was mainly composed of larger thin-walled muscular vessels. The final diagnosis was gastric splenosis with hemangioma.Interventions:After discussion in a multidisciplinary conference, the patient was considered for a GIST resection under gastroscopy. In the process of peeling, the surface of the mucosal, submucosal, muscle layers and the tumor surface were diffusely oozing. The effect of electrocoagulation and hemostasis was extremely poor. Therefore, endoscopic surgery was arrested. After dealing with the patient's family, a combination of laparoscopic-gastroscope double-mirror surgery was decided in accordance with the principle of minimally invasive surgery to preserve the stomach. Owing to several adhesions and concealed the location of tumor, we stopped the double-mirror combination surgery plan. Considering the great possibility of a malignant GIST, we still decided to continue the traditional surgical resection. The tumor was then removed via surgeryOutcomes:The patient was favorable with healing and discharged on postoperative day 10.Lessons:Gastric splenosis with an associated hemangioma is the first well-documented case. Its pathogenesis may be direct implantation. Appropriate medical history taking and Tc-99 m heat-denatured RBC spleen scintigraphy (Tc-99MHDRS) are valuable for its diagnosis; however, pathology is the gold standard. Surgery is a reasonable treatment for gastric splenosis with hemangioma.