Situs inversus totalis is a rare congenital disorder, which is total transposition of thoracic and abdominal organs. Its incidence is 1 in 10,000~50,000 live births. This might be associated with multiple abnormalities such as accessory spleen, asplenia, intestinal malrotation and so on. For this reason, in cases of operation in patients with situs inversus totalis, we need to scrutinize the presence of accompanied anomalies. Moreover, if Dieulafoy gastric bleeding has occurred, vascular anomalies can be accompanied. This 31-year-old male patient with situs inverses totalis was admitted to our hospital for management of UGI (upper gastrointestinal) bleeding. Gastroendoscopy revealed Dieulafoy disease in the upper body of the stomach as the cause of UGI bleeding. Several attempts with endovascular embolization and hemoclips were applied but failed. We performed a suture & ligation of the Dieulafoy lesion as well as total resection of accessory spleen with devascularization of prominently developed vessels around the upper stomach. We report this case with a review of the literature.