Esophageal duplications are congenital abnormalities of the foregut. We present the case of a 33-year-old woman suffering from progressive dysphagia who had surgery for esophageal duplication. The following three criteria define the cystic lesion: an intimate attachment to the esophageal wall, the presence of a smooth muscle coat and a mucosal lining consisting of squamous and/or ciliated respiratory epithelium. Diverticula, bronchogenic cysts and cystic neoplasms have to be considered in the differential diagnosis. Congenital cystic esophageal duplication is a rare cause of dysphagia in adulthood.