A 32-year-old woman presented with shortness of breath and palpitations. Echocardiography and contrast-enhanced computed tomography showed a 4 x 5 cm cyst in the apex of the right ventricle. The cyst was excised on cardiopulmonary bypass. The patient made an uneventful recovery. At the one-year follow-up, she was symptom-free and without recurrence. Different clinical aspects of cardiac hydatid cyst and its surgical management are reviewed.