Kawasaki disease (KD) is an acute vasculitis syndrome of unknown etiology that mainly affects the coronary arteries. The purpose of this study was to assess the agreement between dipyridamole stress technetium-99m tetrofosmin (Tc-TF) myocardial perfusion single photon emission computed tomography (SPECT) and coronary angiography in these patients. Twenty-nine children with KD were included in this study. All of the 29 children also received dipyridamole stress Tc-TF myocardial perfusion SPECT within 1 month of their coronary angiographic studies. The results showed that (1) 89.7% of children had negative coronary angiographic findings without significant coronary stenoses, and 10.3% of children had positive coronary angiographic findings with significant coronary stenosis; (2) 44.8% of children had negative Tc-TF myocardial perfusion SPECT findings without abnormal myocardial perfusion, and 55.2% of children had positive Tc-TF myocardial perfusion SPECT findings with abnormal myocardial perfusion; (3) 44.8% of children had both normal coronary angiographic and Tc-TF myocardial perfusion SPECT findings, and 10.3% of children had both abnormal coronary angiographic and Tc-TF myocardial perfusion SPECT findings; and (4) There was no significant agreement between coronary angiographic and Tc-TF myocardial perfusion SPECT findings. We concluded that poor agreement exists between coronary angiographic and Tc-TF myocardial perfusion SPECT findings with coronary stenoses and abnormal myocardial perfusion in children with KD.