Esophageal intramural pseudodiverticulosis (EIPD) is a rare benign disorder characterized by dilation of the submucosal glands. Its etiology and pathogenesis are largely unknown. So far, less than 10 pediatric cases of EIPD have been reported. The authors present the case of a 12-year-old boy with EIPD that was associated with a cervical esophageal web. He was treated successfully with dilation therapy.