Purpose of review: To review the current literature of primary and secondary central nervous system vasculitis in children, to evaluate the evolving classification and to suggest an approach to diagnosis.

Recent findings: Central nervous system vasculitis in children is a newly recognized inflammatory brain disease, which may develop as a primary condition, or secondary to an underlying systemic disease. Primary angiitis of the central nervous system of childhood (cPACNS) is a reversible cause of severe neurological impairment, including acute ischemic stroke, intractable seizures and cognitive decline. Once clinically suspected, angiography is a key imaging modality. Primary angiitis of the central nervous system of childhood can be divided into large-medium vessel and small-vessel vasculitis, each presenting with distinct clinical and radiological features. Angiogram-negative, small-vessel primary angiitis of the central nervous system of childhood has a broad differential diagnosis that includes both vascular and nonvascular conditions. Recognition of this disease and a thorough diagnostic evaluation is necessary to identify and possibly reverse severe neurological impairment in children.

Summary: This review summarizes the recent data on diagnosis and differential diagnosis of primary and secondary central nervous system vasculitis in children. Increasing recognition of this condition in children allows for greater understanding of the spectrum of disease presentation and differential diagnosis of this reversible cause of severe neurological impairment in children.