Esophageal intramural pseudodiverticulosis is a very rare disease of unclear etiology. The clinical picture is characterized by progressive dysphagia. Because of its frequent association with alcohol abuse and subsequent weight loss, it must be differentiated reliably from esophageal carcinoma. The diagnosis is established by the characteristic detection of multiple intramural contrast accumulations in the barium esophagogram. Additional endoscopic and endosonographic confirmation and histological examination are required to exclude a malignant tumor. Moreover, associated diseases are almost always present and should also be diagnosed by pH-metry, cytology, and esophageal manometry. Good and long-lasting therapeutic success can be achieved by bouginage of the stenosis with concomitant treatment of the associated esophageal diseases. Based on two case reports of patients with this disease, we discuss the unusual association with esophageal hypermotility as well as the symptoms, clinical course, therapy, and pathogenesis of the disease.