Background: Gastroesophageal junction (GEJ) submucosal tumors are comprised predominantly of leiomyomas, followed by gastrointestinal stromal tumors. Methods: Herein we provide a comprehensive review of GEJ submucosal tumors including a systematic review of the literature. Results: Computed tomography, esophagogastroduodenoscopy, and endoscopic ultrasound are the most commonly used diagnostic imaging modalities. Surgical resection via laparoscopy is the most common treatment approach, with simultaneous upper endoscopy utilized in approximately half of the cases. Concomitant fundoplication is performed by certain groups to minimize risk for postoperative leak, gastroesophageal reflux, and esophagitis. Conclusions: Prospective studies evaluating long-term outcomes are needed to help guide treatment.