Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. They frequently occur in the stomach and small intestine, but they rarely occur in the colon and rectum. This is a case report of a patient with a GIST involving the cecum, and acute obstruction of the small intestine. A 47-year-old male patient was admitted to the emergency surgery department with abdominal pain and distension lasting for a few days. Clinical examination revealed tenderness in the right iliac fossa. Routine hematologic testing revealed anemia, and abdominal x-ray multiple air-fluid levels in the small intestine. Based on the clinical findings, we decided to perform an explorative laparotomy. A large cecal tumor was detected. The proximal parts of the small intestine were severely distended. GISTs are more common in the stomach (60-70%) and the small intestine (25-35%), than in the colon, rectum and esophagus. The most frequent complications associated with GISTs are obstructions and hemorrhages. Surgical resection is the primary treatment of GISTs. Radiotherapy and chemotherapy are generally ineffective. Continuous postoperative follow-up is necessary, because most recurrences occur within the first 2 years after complete surgical resection. In the past few years, surgical resection was followed by adjuvant tyrosine kinase inhibitor therapy.