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Isolated cardiac location is an uncommon presentation of echinococcosis (0.5-2%), and involvement of the interventricular septum is even rarer. It may lead to various complications because of rupture and embolization. We report the case of a 26 - year- old man who was diagnosed to have a large inter-ventricular hydatid cyst complicated by both cerebral and coronary embolism. Presentation, management and follow-up of the patient is discussed. This case is of particular interest because of the rarity of septal localization of a hydatid cyst, and the conflict between the severity of the complications that occurred and the absence of correlated symptoms. Keywords: Large hydatid cyst, Interventricular septum, Acute coronary syndrome, Cerebral 19 embolization, Echocardiography, MRI, Surgery