Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors in adults. They frequently occur in the stomach. Gastric GISTs typically present as a gastrointestinal bleed but can sometimes cause obstructive symptoms such as nausea and vomiting. We present a patient with a gastric GIST and liver metastases who during treatment with iminitab therapy presented with an acute gastric outlet obstruction. A computed tomography scan revealed a gastroduodenal intussusception of the gastric GIST. The patient underwent a laparoscopic exploration and resection of the GIST. We reviewed the English language literature of GISTs that presented as a gastroduodenal intussusception and put our case in the context of the previously reported cases. We discuss the diagnostic and therapeutic challenges that arise when treating these patients.