To the Editor: Primary angiitis of the central nervous system (PACNS), is a rare form of vasculitis restricted to the brain and spinal cord. The condition results in diffuse inflammation and destruction of small-to-medium sized blood vessels in the CNS. The clinical symptoms are nonspecific and reflect the diffuse nature of the pathological process. Headache is a common symptom. Other symptoms include cognitive impairment, stroke, transient ischemic attacks, cranial nerve involvement, myelopathy, seizures, and ataxia.1 Apart from reporting that cognitive deficits can be present, to-date no reports of cognitive and behavioral functioning in patients with PACNS have been published. Here, we describe the neuropsychological functioning of a patient with confirmed primary angiitis, at baseline and at 3 and 10 months of treatment with immunosuppressive medication.