BACKGROUND: Cardiac myxomas are rare benign tumors of the heart. Although cardiac myxomas are histologically benign, they may be lethal because of their strategic position. Most cases are sporadic, but rare familial occurrence has been described. PATIENTS AND RESULTS: The left atrial myxoma with cerebral embolism was diagnosed in the 21 year old female and the left atrial myxoma with acromegaly due to pituirary adenoma was subsequently diagnosed in her 19 year old male sibling. The myxoma in the male patient was successfully excised. CONCLUSION: Echocardiography can be used effectively in the diagnosis of atrial myxoma, detection of its possible recurrence, and screening other members of the family.