The circle of Willis is a very important vascular mechanism of protecting against cerebral ischemia, especially when circulation within the main arteries irrigating the brain is somehow impeded. As result of congenital malformation arising early in embryonic development, the fetal-type posterior circle of Willis remains as such during the rest of one's life. Consequently, the posterior cerebral artery (PCA) becomes a branch of the internal carotid artery (ICA), rather than of the basilar artery (BA). Furthermore, the rest of collateral circulation, between the anterior and the posterior regions of the brain, is also negatively affected (e.g., leptomeningeal vessels). The anatomical variant represented by the artery of Percheron (AOP) has its origin on one of the PCAs, supplying singlehandedly both paramedian areas of the thalamus (right and left) and posterior regions of the midbrain. In the present study, we report a case of bilateral thalamic infarction with midbrain involvement, where the correct diagnosis was made retrospectively using computed tomography (CT) scan, magnetic resonance imaging (MRI), diffusion-weighted imaging (DWI) and three-dimensional time-of-flight magnetic resonance angiography (3D TOF MRA).