We report the results of percutaneous transluminal coronary rotational atherectomy (PTCRA) for localized stenosis caused by Kawasaki disease (KD) in children. Five males and a females, aged 5-15 years old (median, 9), underwent PTCRA. The interval from the onset of KD to PTCRA ranged from 5 to 12 years (median, 9). The target vessels were the left anterior descending artery (three patients), the left circumflex (two patients), and the right coronary artery (one patient). The immediate results of PTCRA were successful in all patients, and the mean stenosis degree improved from 89 +/- 10% to 27 +/- 12%. In follow-up coronary angiograms within 1 year, four vessels were restenosed, including two with complete occlusion. A 15-year-old male has had good patency for 4 years after undergoing re-PTCRA for restenosis using a larger burr size. PTCRA is feasible for severe localized stenosis with calcification caused by KD in children and the immediate results are good. However, restenosis often occurred within 1 year after PTCRA in small children and PTCRA is not always appropriate for them. When the use of a larger burr is possible, good patency can be expected and can be maintained by close follow-up and re-PTCRA.