Anterior choroidal artery (AChA) territory infarcts represent the second most common infarct in the territory of the deep perforators of the carotid artery system. The classical pattern of clinical signs attributed to AChA territory infarction is hemiplegia, hemianesthesia, and homonymous hemianopia, often associated with neuropsychological signs. Aphasia, spatial neglect, attention disorder, executive functioning impairment, and delayed memory are reported to be less severe than when due to thalamic or cortical infarctions. The high prevalence of arterial hypertension or diabetes mellitus as an isolated risk factor of stroke in small-sized AChA infarcts suggests that small artery disease is a common etiology of AChA territory infarcts. The clinical syndromes, vascular risk factor profile, presumed etiology of stroke, and prognosis allow consideration of small and large AChA territory infarcts in the differential diagnosis of patients with brain ischemia.