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Cardiac hydatidosis constitutes about 0.5-2% of all hydatid diseases. The interventricular septum is involved in 4% of cardiac hydatidosis cases. We demonstrate the surgical management of a large (76 x 66 mm) septal hydatid cyst in a 20-year-old man. The hydatid cyst was approached through a right ventriculotomy under cardiopulmonary bypass. The germinative membrane was removed in toto. The cavity was then obliterated to prevent recurrence of the cyst. The patient had an uneventful postoperative recovery. Histopathological examination confi