Cricopharyngeal achalasia (CPA), first described in children by Utian and Thomas in 1969, 1 is an uncommon cause of neonatal and pediatric oropharyngeal dysphagia. 2 Although current studies on pediatric CPA are all limited to case reports, the disease has received widespread attention because of its serious consequences such as failure to thrive or sudden death in infants and children. Several strategies have been recommended for the diagnosis and treatment of pediatric CPA. However, the gold standard for its diagnosis and management is still lacking. Herein, we presented a case of a 13-year-old boy with severe dysphagia who was diagnosed with CPA and managed with endoscopic myotomy, trying to deepen the understanding of pediatric CPA.A 13-year-old boy with dysphagia for 10 years was admitted to our hospital. He was born at full-term gestation after an uncomplicated pregnancy, with no congenital abnormalities and unremarkable family history. No feeding problem was observed throughout his infancy. At the age of 3, he developed dysphagia without any apparent causes, accompanied by coughing during water intake, hoarseness, and immediate postprandial vomiting. He underwent endoscopic