Achalasia is a rare but debilitating condition that affects approximately 1 to 2 in 100,000 persons. Although exact causes are unknown, achalasia may have its inception in familial, infectious, or autoimmune pathology. Regardless of the cause, patients with achalasia experience dysphagia, esophageal aparistalsis, regurgitation of food and fluids, and a range of other symptoms that impact quality of life for these patients. In this article, the authors explain the etiology and symptomatology of achalasia and present diagnostic and treatment options for practitioner consideration.