Gastrointestinal stromal tumor is a rare pathology. GISTs account for 0.3-1% of all tumors of the gastrointestinal tract. At the same time, this type of cancer is the most common, malignant, non-epithelial tumor of the gastrointestinal tube. Over 90% of GISTs are found in the stomach and small intestine. This cancer usually develops without characteristic clinical symptoms and is diagnosed incidentally. This clinical situation, in which the first symptom of a GIST-pattern tumor includes a fully-symptomatic, non-complicated umbilical hernia, is an unprecedented anomaly. This work presents a case report of a 77-year old female patient undergoing elective surgery, in which the contents of the hernial sac included a stromal tumor. Disseminated, multi-focal progression of the disease was found intraoperatively. Postoperative histopathology and immunohistochemistry revealed a gastrointestinal stromal tumor GIST of the spindle cell type, showing a CD-117, CD-34, SMA expression with possible starting point in the small intestine.