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Eventration of the diaphragm is a rare entity, characterised by abnormal elevation of a dome of diaphragm. In this condition, the diaphragm is composed of fibrous tissue with few or no interspersed muscle fibres. Eventration can be congenital or acquired. Congenital eventration results from inadequate development of muscles or absence of phrenic nerve. The common cause of acquired eventration is injury to the phrenic nerve from traumatic birth injury or surgery for heart disease. The perioperative anaesthetic management of diaphragmatic eventration along with ventricular septal defect with severe pulmonary hypertension makes this case both challenging and unique.