IntroductionCardiac hydatid disease is uncommon and occurs in 0.5 to 2% of patients with hydatidosis. Isolated intrapericardial hydatid cystic disease is extremely rare. ObservationWe report the case of a young woman with cardiac compression due to multiple primary intrapericardial hydatid cysts. Since 1 year, she had gradual general health deterioration including dyspnoea, sweats and weight loss of 8kg. A widening of the mediastinum was observed on chest X-ray. The CT-scan, echocardiography and the dynamic IRM showed multiple mediastinal cysts with mass effect on the heart and main pulmonary artery. The size of the main pulmonary artery was reduced to 5 mm in diameter and the right upper pulmonary vein was nearly closed by posterior cysts. The right and left ventricular ejection fractions were estimated at about 34%. A complete resection of the cysts was performed by sternotomy. The surgical procedure was technically difficult because of major local inflammatory process. The postoperative outcome after an initial pulmonary embolism event was finally favourable. ConclusionHydatidosis can lead to severe cardiac involvement. These rare forms of hydatid cystic disease must be known even in non endemic regions by surgeons because of increasing mobility of the world's population.