Isolated infundibuloarterial inversion is a rare conotruncal cardiac anomaly characterized by a leftward and posterior aortic valve in the face of ventriculoarterial concordance. It has previously been described only in cases associated with severe additional defects of substantial hemodynamic significance. We present a case of isolated infundibuloarterial inversion with a small, hemodynamically insignificant ventricular septal defect. The nature of this lesion, diagnostic methods of imaging, and features aiding its discrimination from the more commonly recognized anatomically corrected malposition of the great arteries are discussed.