The Artery of Percheron (AOP) is an uncommon anatomic variant that provides arterial supply to the paramedian region of the thalami and bilaterally to the rostral part of the midbrain; it is a solitary arterial trunk that branches from a proximal segment of the posterior cerebral artery (PCA). Although AOP infarction results in a characteristic pattern of ischemia-namely bilateral paramedian thalamic infarct with or without midbrain involvement-it may cause diagnostic difficulties due to the variety of its clinical presentations and wide differentials, as well as its small diameter and the difficulty of obtaining visualization through diagnostic imaging. Early neuroimaging of AOP infarction and correct diagnosis are mandatory for early initiation of the appropriate treatment and better patient outcomes. In this study, we discuss imaging the patterns of AOP infarction and its differentials and clinical presentation.