BACKGROUNDGastrointestinal schwannomas are slow-growing benign mesenchymal neoplasms that originate from Schwann cells of the nerve sheath of Auerbach´s plexus or less frequently from Meissner´s plexus. The main differential diagnosis of gastric schwannomas are the gastrointestinal stromal tumors (GISTs), which are classified by their immunohistochemistry. The treatment of choice for gastric schwannomas is surgery where laparoscopy plays an important role. Wedge resection, subtotal or total gastrectomy can be done. In its counterpart, esophageal schwannomas are benign tumors of the esophagus that are very uncommon since they comprise less than 2% of all esophageal tumors. The main differential diagnosis is the leiomyoma which corresponds to the most common benign esophageal tumor, followed by GIST. The treatment consists on tumoral enucleation or esophagectomy. AIMTo review the available literature about gastrointestinal schwannomas; especially lesions from de stomach and esophagus, including diagnosis, treatment, and follow up, as well as, reporting our institutional experience. METHODSA systematic review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyzes guidelines. The following databases were used for reviewing process: PubMed, Ovid, MEDLINE, and Scopus. Only English language manuscripts were included. All gastrointestinal schwannomas specifically located in the esophagus and stomach were included. Cases that did not report long-term follow-up were excluded. RESULTSGastric localization showed a higher prevalence in both, the literature review and our institution: 94.95% (n = 317) and 83% (n = 5) respectively. With a follow-up with disease-free survival greater than 36 mo in most cases: 62.01% (n = 80) vs 66.66% (n = 4). In both groups, the median size was > 4.1 cm. Surgical treatment is curative in most cases. CONCLUSIONSchwannoma must be taken into account in the differential diagnosis of gastrointestinal mesenchymal tumors. It has a good prognosis, and most are benign. A disease-free survival of more than 36 mo can be achieved by surgery.