Infarction in the territory of the anterior choroidal artery (AchA) has been the subject of several recent reports. To the classical clinical syndrome of hemimotor, hemisensory, and visual field deficit has been added hemiataxia acute pseudobulbar mutism, pure motor and pure sensory syndromes and disorders of higher cortical function. The definition of anatomic and clinical correlates to AchA stroke is aided by CT-MRI findings and reveals an unexpected superior extension of infarct to include the periventricular caudate nucleus and inferior corona radiata. Prognosis depends upon unilaterally, bilateral strokes having often a fatal outcome. Etiology and treatment may be intimately related to hypertension.