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Achalasia is a rare motor disorder of the esophagus known for since several centuries ago. It was first described in 1674 by Sir Thomas Willis, but the term “achalasia” was coined only in the twentieth century to indicate failure of lower esophageal sphincter relaxation as a cause of the clinical manifestations of this disease. Despite increasing understanding of pathophysiology of achalasia, its etiology remains largely unknown. Treatment options (drugs, endoscopic therapy, surgery) aim to alleviate symptoms and to improve quality of life, as the underlying neuronal disorder cannot be corrected. Therefore, a complete diagnostic workup (radiology, endoscopy, manometry) is a key element for the choice and the success of the treatment and it should evaluate disease features and stage in each case and take in account patient’s clinical conditions and preference. Given the low incidence of the disease, there are only a few randomized controlled trials to define the optimal therapeutic strategy and in many clinical settings, management of achalasia is influenced by local expertise and availability of each procedure. Mininvasive surgery seems to achieve the best short and long-term clinical outcomes and is considered by many authors the treatment of choice.