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Introduction: Epiphrenic diverticulum is a rare disease caused by mucosa and submucosa herniation through the muscular layers of the esophageal wall. This study presents a case of a patient with a symptomatic epiphrenic diverticulum treated with surgery under endoscopic assistance. A review of the literature on this unusual condition was conducted, focusing on the pathogenesis, presentation, and surgical challenges. Methods: A 75-year-old woman who underwent an esophagogastroduodenoscopy (EGD) after experiencing worsening dysphagia for food and liquids. The EGD revealed a large epiphrenic diverticulum 36 cm from the upper incisor teeth. The presence of a diverticulum was verified by barium swallow, which revealed a 6 cm diameter epiphrenic diverticulum on the right side of the esophagus. Results: Patient underwent laparoscopic diverticulectomy associated with Heller's myotomy and anterior partial fundoplication. A gastroscope was placed intraoperatively to calibrate the esophagus to prevent stenosis during diverticulectomy, and it was also used to check the integrity of the esophageal wall. The patient tolerated clear liquids on postoperative day 2. Postoperative course was complicated by right pleural effusion and fever managed with antibiotics and pleural drainage. Conclusion: Epiphrenic diverticulectomy in conjunction with management of the underlying motor dysfunction and avoidance of gastroesophageal reflux disease is an effective procedure. Laparoscopy is considered the approach of choice for the majority of patients. Endoscopic assistance during surgery can help the identification of the diverticulum and verify the integrity of the staple line.