Occlusion of the middle cerebral artery in a 16-year old woman reveale
d a left atrial myxoma associated with a mucocutaneous lentiginosis. T
wo-dimensional transthoracic or transoesophagal echocardiography shoul
d be performed in all patients with stroke and lentiginosis to look fo
r cardiac myxoma. After treatment of the cardiac tumor, echocardiograp
hy should be repeated at regular intervals to detect a recurrence of t
he myxoma. As this cardio-cutaneous syndrome is a familial disorder wi
th autosomal dominant inheritance, examination of all family, members
by echocardiography is recommended.