Gastrointestinal stromal tumors (GISTS) are rare, but potentially aggressive tumors. GISTS are generally found in the stomach or small intestine and less commonly in the colon, rectum, or an intra-abdominal sites but have rarely been documented in the esophagus. GISTS were definded as the most common mesenchymal tumors of the gastrointestinal tract for which there is incomplete understanding of their lineage, while their relationship with differenciated. We reported a very rare case of GISTS of lower esophagus in a 60-year-old woman with relevant literature review.