Gastrointestinal stromal tumors (GIST) are rare tumors of the digestive tract. Appendiceal GISTs are very rare, accounting for around 0.1% of the total number of cases. The central elements in the pathogenesis of gastrointestinal stromal tumors are represented by KIT (proto-oncogene) and PDGFRA (platelet derived growth factor receptor) mutations. The tumor can develop in any portion of the vermiform appendix. GISTs are associated with a wide variety of symptoms and complaints. A large part of these tumors is detected during necropsies. Tumors that are smaller than 2 cm usually do not have symptoms and are detected incidentally following exploratory interventions, endoscopic or imaging investigations. About 95% of GISTs stain strongly for CD117 antigen. DOG – 1 is a highly sensitive marker for the diagnosis of GIST and has a higher degree of specificity than CD117, but is not pathognomonic. The standard treatment for GISTs is surgical resection with microscopically negative resection margins.