Any echocardiographic study of two children with hypothyroidism demonstrated the presence of asymmetric septal hypertrophy. One child died aged 11 months, and pronounced thickening of the interventricular septum was confirmed at necropsy. There was also hypertrophy of the left ventricular free wall. Histological examination showed only slight muscle fibre disarray, but there was striking vacuolation and hypertrophy of muscle fibres. In the second case, a child aged five years, the asymmetric septal hypertrophy disappeared after 18 months' treatment with L-thyroxine. Furthermore, other indices of myocardial function also returned to normal. The mechanism producing asymmetric septal hypertrophy in hypothyroidism is unknown. These are the youngest cases in which this association has been reported.