A patient with hypertrophic cardiomyopathy (HCM) who presented with preexcitation pattern on the surface ECG suggestive of the Wolff-Parkinson-White (WPW) syndrome is described. Intracardiac electrophysiological study revealed a fixed anomalous QRS complex and a short-fixed His-ventricular interval indicating a fasciculoventricular Mahaim fiber. As this specific form of accessory connection does not cause reentrant tachycardias, no treatment was required. It is important to distinguish this entity from atriofascicular or nodoventricular Mahaim fibers or the WPW syndrome in patients with HCM showing a preexcitation pattern in the surface ECG, as these may cause life-threatening arrhythmias in this patient population.