Diffuse esophageal leiomyomatosis is a rare cause of dysphagia. There is frequently a chronic inflammatory infiltrate, but here we present the first reported case in association with a dense infiltration of eosinophils and mast cells confined to the affected muscularis propria. This was successfully managed by long esophageal myotomy, with resolution of dysphagia and no symptomatic recurrence after 3 years.