Abstract: Objective To discuss the probable pathogenesis, clinical manifestations, diagnostic and treatment methods, and prognosis of intramural esophageal dissection (IED), in order to improve diagnostic and therapeutic levels for IED. Methods We retrospectively analyzed the clinical data of one patient suffering from circumferential intramural dissection of whole thoracic esophagus with inflammation of false lumen and localized esophageal perforation treated in the First People’s Hospital Affiliated to Shanghai Jiaotong University in February 2010. The 56 years female underwent right exploratory thoracotomy through a standard posterolateral incision in the fifth intercostal space with the whole diseased esophagus resected and the stomach anastomosed through retrosternal tunnel to the cervical intact esophagus in the left neck. Case reports with integral clinical data in recent 10 years’ literature were reviewed through PubMed searching system with the keyword being intramural esophageal dissection or intramural esophageal hematoma. Results The patient was finally cured by whole thoracic esophagectomy and discharged at postoperative day 14. Halfyear followup result was satisfactory. Thirteen cases with integral clinical data were reviewed. The major manifestations were mainly chest and dorsal pain, odynophagia and dysphagia, and occasional hematemesis. Diagnosis was mainly based on esophagography, endoscope and CT. Twelve patients were cured or remitted after conservative therapy, endoscopic therapy or surgical therapy. One patient died after surgical exploration. Conclusions IED is arare disease, and esophagography, endoscope and CT are important diagnostic methods. IED is widely regarded as benign process which responds to conservative managements and endoscopic treatments. However, in some severe cases, we suppose that removal of the diseased esophagus is more reliable.