Purpose: Abnormal myocardial perfusion may be caused by ventricular preexcitation, but its location, extent, severity and correlation with accessory pathway (AP) are not established. We evaluated perfusion patterns on myocardial perfusion SPECT and location of AP in patients with WPW (Wolff-Parkinson-White) syndrome. Materials and Methods: Adenosine Tc-99m MIBI or Tl-201 myocardial perfusion SPECT was performed in 11 patients with WPW syndrome. Perfusion defects (PD) were compared to AP location based on ECG with Fitzpatrick's algorithm or electrophysiologic study and radiofrequency catheter ablation. Results: Patients had atypical chest discomfort or no symptom. Risk of coronary artery disease (CAD) was below 0.1 in 11 patients using the nomogram to estimate the probability of CAD. Coronary angiography was performed in 4 patients (mid-LAD 50% in one, normal in others). In 4 patients, AP localization was done by electrophysiologic study and radiofrequency catheter ablation (RFCA). Small to large extent (11.0¡¾8.5%, range:3~35%) and mild to moderate severity (-71¡¾42.7%, range:-217~-39%) of reversible (n=9) or fixed (n=1) perfusion defects were noted. One patient with right free wall (right lateral) AP showed normal. PD locations were variable following the location of AP. One patient with left lateral wall AP was followed 6 weeks after RFCA and showed significantly decreased PD on SPECT with successful ablation. Conclusion: Myocardial perfusion defect showed variable extent, severity and location in patients with WPW syndrome. Abnormal perfusion defect showed in most of all patients, but it did not seem to be correlated specifically with location of accessory pathway and coronary artery disease. Therefore myocardial perfusion SPECT should be interpreted carefully in patients with WPW syndrome.(Korean J Nucl Med 39(1):9-14, 2005)