Achalasia is a chronic gastrointestinal disorder characterized by increased esophageal sphincter tone and dysmotility that causes worsening dysphagia. While this condition usually affects the lower esophageal sphincter, we present a rare case of upper esophageal sphincter (UES) achalasia of unknown etiology in a female in her sixth decade of life. This was managed via UES myotomy but was complicated by esophageal perforation and severe post-operative stenosis. Consequently, the patient was referred to gastroenterology and treated over the course of two months with six endoscopic dilatations and glucocorticoid injections. Few cases of idiopathic UES achalasia have been described to date.
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