Background:Congenital hemangioma of the diaphragm is an extremely rare disease in childhood.Methods:We report a newborn presenting with progressive respiratory distress and massive right hydrothorax due to congenital diaphragmatic hemangioma, requiring sustained ventilation support and chest drainage. The angiography revealed that the giant diaphragmatic hemangioma was supplied by the right internal thoracic, inferior diaphragmatic, and intercostal arteries. The selective embolization of the main feeding vessels was successfully achieved using the Embosphere particulates.Results:The clinical long-term follow-up demonstrated the resolution of the symptoms, pleural effusion, and nearly complete regression of the hemangioma at 18 months of age.Conclusion:Transcatheter embolization can provide an efficient therapy for symptomatic diaphramatic hemangioma.