The purpose of this article is to review the clinical features, pathophysiology, diagnosis, and management of patients with diffuse esophageal spasm (DES). The PubMed database was searched with a focus on recent publications, using keywords "DES," plus "epidemiology," "prevalence," "diagnosis," "pathogenesis," "calcium channel blocker," "nitrates," "botulinum toxin," "antidepressants," "dilation," and "myotomy." The reference lists of papers identified in the initial PubMed search were reviewed for further relevant publications.