Benign tumors of the esophagogastric junction are rare and mostly found incidentally during endoscopic or radiological procedures which are often performed for unrelated reasons. Epithelial lesions must be distinguished from subepithelial findings, where the latter are far more common and mostly represent benign leiomyomas, especially in the esophagus. Some benign tumors, such as small gastrointestinal stromal tumors, have malignant potential and must therefore be distinguished from completely benign lesions. Epithelial tumors can be diagnosed by standard endoscopic biopsies, whereas tissue acquisition of subepithelial lesions is more challenging. For this purpose endosonography is a valuable tool even before tissue acquisition or resection is performed. Endosonography can identify the gastrointestinal wall layer from which the lesion arises and gives additional information by identification of characteristic echographic features. Endoscopic resection techniques are used to remove those tumors that originate from the mucosa or submucosa. Some authors have even described resection of tumors originating from the deep muscle layer, but these techniques have a significant risk of perforation. Several surgical techniques are available for complete resection ranging from laparoscopic enucleation to combined endoscopic laparoscopic (rendezvous) techniques and abdominothoracic approaches. The current diagnostic and therapeutic challenges regarding benign tumors of the esophagogastric junction are outlined in this review article.