We present here a 44-year-old male patient with hydatid disease who was referred to our hospital due to dyspnea and chest pain for the last 2 month before admission. Using echocardiography and contrast-enhanced computed tomography the heart hydatid was diagnosed. However, hydatid disease of the interventricular septum is rare; particularly, the involvement of mitral apparatus with mitral regurgitation (MR) is an exceptionally rare presentation. Early diagnosis and an integrated treatment strategy are crucial. Surgical excision was performed and the patient had an uneventful recovery and follow-up at 3 months.