Hydatid disease is a common health problem in the sheep-farming countries of the Mediterranean, caused by infection with Echinococcus granulosus. The common sites are the liver and lungs. Cardiac hydatidosis is rare. Herein, we report a case of interventricular septum hydatid cyst with lung involvement in a four-year-old child. Cardiac hydatid cysts should be resected surgically even in asymptomatic patients since delayed diagnosis and treatment can lead to fatal outcomes. In spite of its rarity, the combination of cardiac and pulmonary hydatidosis can be managed with a single operation.
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