Ectopic spleen is a condition in which the spleen does not sit in the left hypochondrium but has an atypical, often pelvic, location. We report the case of a 35-year-old patient suffering from chronic abdominal pain for 11 years associated with an episode of hematemesis, pollakiuria and dysuria, with anemia and notion of multiple transfusions. Abdominal ultrasound found an ectopic spleen in the pelvic position, enlarged in size, with multiple collateral venous circulations and extensive partial thrombosis of the splenic vein. A total splenectomy by midline laparotomy was performed. Perioperative exploration found a bulky pelvic spleen with a long and tortuous splenic pedicle, involuted in 3 turns without splenic infarction. When the splenic artery is clamped, the colonic and gastric varicose veins lose half their diameter, so the decision is made to perform total splenectomy. Post-splenectomy antibiotic prophylaxis and vaccination was administered. The follow-up was favorable, with a follow-up of 4 years. The patient was then lost sight of.