Central nervous system localizations of sarcoidosis remain rare. The brain, mainly the posterior fossa, or the spinal cord, mainly cervical, may be involved. We report the case of a white female presenting a stroke of the head of the caudate nuclei with spontaneous hemorrhage transformation related to and associated with rare encephalic and spinal cord sarcoidosis. The magnetic resonance angiography (MRA) of the intracranial vessels showed images of vasculitis. Treatment of the neurologic localizations was difficult.