Objective To improve recognition for pericaridiac lymphangioma and avoid inappropriate intervention. Methods Pericardial lymphangioma confirmed by pathological histology in a 39-year-old patients was retrospectively analyzed. Results A woman without past medical history was admitted to our hospital for chest discomfort and fatigue on exertion. A large tumoural mass located in the posterolateral wall of the left ventricle was visualized by trans-thoracic echocardiogram. Subsequent chest contrast-enhanced CT showed an unenhanced cystic mass covering the surface of left ventricle,which simultaneously encompassed the left anterior descending artery and concomitant vein(Fig 1). The patient underwent surgery via left thoracotomy under general anesthesia. After opening the parietal pericardium,multicystic dark-purple mass measuring 10.0cm×8.0cm×6.0cm was seen and originated in the left-ventricular cardiac wall with a broad base(Fig 2a). After completing extensive resection and myocardial suture, cardiac arrest happened three times and was successfully rescued by cardiac massage and electrical defibrillation. The resected specimen of the tumor revealed a uniformly thin-walled, multicystic lesion with a smooth external surface(Fig 2b) and light yellow serous content. Histological analysis showed a cystic tumor with a predominantly lymphatic vascular component, lymphoid follicles and mature adipose tissue. The final diagnosis was cardiac lymphangioma. The postsurgical recovery of the patient was uneventful and she was in good condition on the followup of 16 months(Fig 3).