The artery of Percheron (AOP), which is a rare anatomical variant, is characterized by a solitary common arterial trunk arising from the posterior cerebral artery (PCA). Occlusion of this artery is rare and may lead to a bilateral paramedian thalamic infarction with or without midbrain involvement. The AOP is a rare anatomical variant that is present in 4–12% of the population. The AOP stroke represents 0.1–2.0% of ischemic strokes and 4–18% of thalamic infarcts. This case series describes the varied clinical manifestations and the imaging findings of AOP infarct. The symptoms noted in the series are altered mental state, hypersomnolence, vertical diplopia, memory disturbances, hemiparesis, and ataxia. The magnetic resonance imaging (MRI) findings showed bilateral paramedian thalamic infarct with or without the involvement of the midbrain. The complexity and polymorphism of AOP stroke semiology explain why bilateral thalamic infarction is often misdiagnosed, lately detected, or even not detected. It is a real diagnostic challenge for clinicians to detect this condition in a timely fashion. Its diagnosis and treatment may be delayed because of the wide spectrum of its clinical features.