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In 1988 a 26 year old man came to our department because of cardiomegaly found at a routine chest x ray. He had no history of cardiac disease and was asymptomatic. Basic electrocardiography was normal. Echocardiography revealed normal cardiac chambers and valves and a discrete pericardial effusion; therefore, the patient underwent diagnostic pericardiocentesis. The fluid was milky with a high cholesterol, triglyceride, and protein content (1.68 mmol/l, 48.58 mmol/l, and 50.0 g/l, respectively), while a large chylomicron band was present at electrophoresis. The presence of lung or mediastinum abnormalities was excluded by thoracic computed tomography. A lymphangiography showed an open thoracic ductus as far as the fifth thoracic vertebra, then the contrast spread towards the anterosuperior portion of the thoracic wall, shaping small sacculations and thin lymphatic ways. Thus, an idiopathic chylopericardium owing to mediastinal lymphangiectasias was diagnosed. The patient was kept on a medium chain triglyceride diet and was not surgically treated. At nine years’ follow up the pericardial effusion had completely disappeared.