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Diaphragmatic hernia is primarily congenital in origin and has potentially devastating pulmonary complications. Acquired diaphragmatic hernia as a complication of hydatid disease remains a rare clinical entity. Retroperitoneal hydatidosis, in particular is an exceptionally rare cause behind a similar presentation. This paper aims to present the first case of acquired diaphragmatic hernia likely caused by eroding retroperitoneal hydatid cysts and provide a succinct literature review regarding the causative association between hydatid dis