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Congenital diaphragmatic malformations include congenital diaphragmatic hernia (CDH) and eventration of the diaphragm. The clinical presentation is variable, and the prognosis depends on multiple factors. The coexistence of CDH and diaphragmatic eventration in the same patient is extremely unusual and has not been reported previously in the literature. We report this rare association in a 3-month-old male infant who was managed successfully by minimally invasive approach.