Primary central nervous system vasculitis (PCNSV) is a rare cause of ischemic stroke and is considered idiopathic in most cases. PCNSV can present with a wide variety of neurological manifestations and should be considered in the differential diagnosis of ischemic stroke, particularly if the neurological deficit cannot be explained by the affected vascular area or when it is multifocal. The diagnosis of PCNSV is relevant because the required therapy differs from the treatments commonly used for frequent ischemic strokes.We report the case of a 64-year-old woman admitted for an ischemic stroke with a right frontal cortico-subcortical ischemic lesion. The etiological investigation documented multiple intracranial arterial stenoses. Secondary causes of central nervous system vasculitis were excluded. The patient refused a brain biopsy, and corticosteroid therapy was initiated due to high suspicion of PCNSV, supported by findings from transcranial Doppler ultrasound and brain magnetic resonance angiography. The patient had a positive clinical outcome and did not have any recurrences while under therapy.This case raises awareness of the importance of considering PCNSV in the differential diagnosis of ischemic stroke. It emphasizes the importance of promptly initiating therapy to minimize PCNSV-associated complications.