Occlusion of the middle cerebral artery in a 16-year old woman revealed a left atrial myxoma associated with a mucocutaneous lentiginosis. Two-dimensional transthoracic or transesophageal echocardiography should be performed in all patients with stroke and lentiginosis to look for cardiac myxoma. After treatment of the cardiac tumor, echocardiography should be repeated at regular intervals to detect a recurrence of the myxoma. As this cardio-cutaneous syndrome is a familial disorder with autosomal dominant inheritance, examination of all family members by echocardiography is recommended.