Preoperative diagnosis of Gastrointestinal Stromal Tumors (GISTs) in the small intestine is often delayed until complications such as hemorrhage, bowel obstruction or perforation develop. Such GISTs are usually asymptomatic and tumor perforation is an uncommon clinical presentation. In this report, we review the diagnosis, pathology and treatment of a 32-year-old male with a perforated GIST in the jejunum who presented with symptoms of acute abdomen. Abdominal computed tomography (CT) showed a ruptured huge tumor with inhomogeneous density in the small intestine. The patient underwent a complete tumor excision and jejunal segmental resection. A histopathological examination of the tumor confirmed that it was a GIST. Postoperatively, the patient received medical treatment, using oral Imatinib 400 mg daily for 10 months without any signs of disease recurrence. Prognosis is worse in ruptured GISTs and in these cases, complete surgical resection of the tumor must be followed by adjuvant therapy with Imatinib.