Esophageal achalasia is a primary esophageal motility disorder characterized by the absence of esophageal peristalsis and failure of the lower esophageal sphincter to relax in response to swallowing. This article reviews the most clinically relevant aspects of diagnosis and management of patients with achalasia, focusing on the several treatment modalities available. At present, laparoscopic Heller myotomy with partial fundoplication is considered the gold standard for the treatment. Endoscopic procedures such as endoscopic botulinum toxin injection and pneumatic dilatation should be considered as primary treatment modalities only in frail patients. Peroral endoscopic myotomy is a new approach with promising short-term results.