Objective Primary cardiac tumors in children are rare. Myxomas are the third most common tissue type behind rhabdomyomas and fibromas. The authors report a clinical case highlighting myxoma as an important cause of embolic events and cerebrovascular disease in children and adolescents.

Methods Review of clinical data.

Results A 12-year-old girl presented with sudden dizziness and right sided paresthesia of the lips and of the right hand fingers. She underwent neurological evaluation and the brain magnetic resonance suggested demyelinating lesions or vasculitis. Other laboratory and imaging studies were made to clarify the clinical situation. The echocardiogram showed a left atrial tumor that was surgically removed without complications. Microscopic examination was compatible with cardiac myxoma.

Conclusions This case emphasises the need for cardiac evaluation and the importance of ruling out an embolic event of cardiac origin in the presence of neurological manifestations of cerebrovascular disease, even when faced with inconclusive brain imaging studies and without any previous evidence of cardiac disease. Prompt clinical recognition and operative removal are essential for the prevention of serious complications.