Although rarely, echinococcosis might present cardiac involvement, with cysts growing inside myocardial structures of arising adjacent to heart. A careful differential diagnosis with other mass formations and rare cardiac tumours is necessary, whenever there is a clinical and radiological suspicion. Imaging studies and serology will establish diagnosis. A multidisciplinary approach is warranted in all cases, with surgical intervention being unavoidable in most settings. Patients generally present with chest pain and dyspnoea. Cases need a close follow up of their postoperative course, while being treated appropriately with albendazole (or mebendazole) for prevention of recurrences.