Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the GI tract, they have a wide spectrum of clinical manifestations, and a small proportion of them present as an acute abdomen due to obstruction, perforation, or hemoperitoneum. We present a case of a perforated GIST of the Meckel's diverticulum and review the current literature on perforated GISTs. We ran a search on Pubmed and Google Scholar with the following terms Bperforated GIST,^Bperforated Meckel's diverticulum,B GIST with peritonitis,^and BGIST in Meckel's diverticulum.^After selecting the relevant articles, we tabulated our results and analyzed the data. Out of the 45 cases, 32 (71.1 %) were male and 13 (28.8 %) were female. Out of the 45 reported cases, 41 (91.1 %) were from the small intestine, 2 (4.44 %) cases from the stomach, and 2 (4.44 %) from the small bowel. In 15 (33.33 %) cases, the site of perforation was the Meckel's diverticulum (MD). Forty-year-old male, presented with features of peritonitis localized to the right iliac fossa, was diagnosed as acute appendicitis and subjected to a diagnostic laparoscopy. Intraoperatively, a perforated Meckel's diverticulum was found, which on histopathological examination contained a low-grade GIST. Our literature search revealed that in reported cases of perforated GISTs, there is a slight male preponderance. Small bowel appears to be the most common site for a perforated GIST, and a significant proportion (30.23 %) arise from the Meckel's diverticulum. And most importantly, the commonest tumor to cause perforation of a Meckel's diverticulum is a GIST.