Achalasia is the best characterized primary esophageal motility disorder of the esophagus and typically presents with absent peristalsis of the esophageal body and a failure of the lower esophageal sphincter to relax upon swallowing on manometry, associated with progressively severe dysphagia, regurgitation, aspiration, chest pain, and weight loss. The diagnosis is suggested by barium swallow and endoscopy, and confirmed by manometry. As there is no curative treatment for achalasia, treatment is confined to disruption of the lower esophageal sphincter to improve bolus passage. Treatment modalities available for this purpose include pneumatic dilation, laparoscopic Heller myotomy and since recently peroral endoscopic myotomy or POEM. In this review, we will discuss the current diagnosis, management, and treatment options of achalasia.