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The esophagus is a crucial organ in the digestive system, and unusual anatomical variations can significantly affect the management of esophageal conditions. These variations, which can be congenital, acquired, or pathology-related, present unique challenges for both esophageal surgery and endoscopic procedures. Congenital anomalies such as esophageal atresia, tracheoesophageal fistula, and congenital stenosis, along with esophageal diverticula (Zenker’s, traction, and epiphrenic), vascular anomalies like esophageal varices, and motility disorders such as achalasia, can complicate diagnosis and treatment. These variations necessitate precise preoperative imaging and tailored surgical approaches to prevent complications such as bleeding, perforation, or poor functional outcomes. Understanding these uncommon anatomical variations is essential for improving the success of esophageal surgeries and endoscopic interventions, ultimately optimizing patient care.