Situs inversus totalis (SIT) is very rare autosomal recessive condition, and patients with SIT have complete mirror image reversal of the thoracic and abdominal viscera. There have been no case reports of esophageal bezoar in a patient with situs inversus totalis. Bezoars are retained concretions of indigestible foreign material, including food material, vegetable material and hair, and they are usually founded in the stomach, small intestine and rectum. Esophageal bezoars are very rare, but they are known to occur in patients with anatomical defects or esophageal motility disorders. The treatment of esophageal bezoar is usually based on endoscopic fragmentation and extraction, dissolution with papain, cellulose, pancreatic enzyme and/or Coca cola. We report here on a case of an endoscopically treated primary esophageal bezoar in a patient with situs inversus totalis, and the patient experienced no complications from the treatment. (Korean J Gastrointest Endosc 2010;41:16-20)