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An intracardiac haemangioma with papillary endothelial hyperplasia (PEH) and liver involvement has not been previously reported in the English literature. This report describes a 65 year old man with a left ventricular haemangioma with PEH coexistent with multiple nodular hepatic haemangiomas. Transthoracic and transoesophageal echocardiography identified a large tumour in the left ventricular cavity with a pedicle connected to the apex. Abdominal sonography also identified multiple hyperechoic hepatic tumours. Magnetic resonance imaging showed hypervascularity of both the cardiac and hepatic lesions. The left ventricular tumour was totally resected and the liver nodules were biopsied. Tissue pathological study showed that both the left ventricular tumour and liver lesions were haemangiomas with PEH. The patient was discharged without complications postoperatively.