Summary:Echinococcal involvement of the heart is a rare occurrence. We report a case of a 46-year-old man with a four-month history of exertional dyspnea and symptoms of atypical angina pectoris, who had a hydatid cyst located in the interatrial septum as well as in the right upper pulmonary lobe. The diagnosis was made by echocardiography, X-ray, and computed tomography. After surgical resection the cyst was opened, its contents were drained, and membranous structures were completely removed. Postoperative albendozol treatment yielded a definite cure with the relief of all symptoms one year postoperatively.