This article reviews the diagnosis and treatment of achalasia, a rare esophageal motility disorder characterized by absent peristalsis and failure of the lower esophageal sphincter (LES) to relax. Various treatment options including management with sublingual nitrates or calcium channel blockers, injection of the LES with botulism toxin, pneumatic dilation of the LES, and pneumatic dilation are discussed. Laparoscopic Heller myotomy is minimally invasive with incumbent low morbidity and mortality rates, and combined with a partial fundoplication is a durable, safe, and effective treatment option for patients with achalasia.