Esophageal gastrointestinal stromal tumors (GISTs) are extremely uncommon, representing approximately 5% of GISTs with the majority of esophageal GISTs occurring at the esophagogastric junction (EGJ). The treatment options available for these GISTs are fairly controversial. Many different options are nowadays at our disposal. From surgery to the target therapies we have the possibility to treat the majority of GISTs, including those which are defined as unresectable. The EGJ GISTs represent a stimulating challenge for the surgeon. The anatomical location increases the possibility of post-operative complications. As the role of negative margins in GIST surgery is still controversial and the efficacy of target therapy has been demonstrated, why not treat EGJ GISTs with enucleation and, where indicated, adjuvant target therapy?