A gas trointes tinal s tromal TUMOR ((GIST)) is the mos t common intramural mesenchymal TUMOR of the gas trointes tinal tract. Their metas tasis to extragas trointes tinal organs is rare, especially the spleen. The patient was a 52-year-old man who, with the diagnosis of peritonitis, underwent laparotomy emergently. At the time of surgery, an extensive TUMOR was seen in the s tomach, esophagus, and jejunum, which had metas tases to the spleen. Total gas trectomy, esophagojejunos tomy, and splenectomy were performed. The morphological s tudies showed a spindle-cell type of (GIST) that were positive for CD117 and Discovered on (GIST)-1 (DOG1). (GIST) was diagnosed as high-risk and treated with imatinib. In follow-up, he died 5 months after discharge. Here, we reported a case of (GIST) synchronous with splenic metas tasis. Surgeons mus t alert for possible metas tases associated with (GIST)s, clinical presentation, diagnosis, treatment, and pathological classification.