With the advent of magnetic resonance imaging as a rapid and accurate way to diagnose arterial ischemic stroke, cerebrospinal fluid assessment is rarely performed, unless infectious or inflammatory processes are obvious. Recent advances in the understanding of the pathophysiology of childhood stroke have implicated a growing list of discrete or occult infectious and inflammatory conditions which may involve intracranial arteries and neighboring structures. Cerebrospinal-fluid assessment may allow the detection of markers identifying processes (including infectious, inflammatory, metabolic, and traumatic) potentially involved in cerebral vasculopathy and stroke. The analysis of cerebrospinal fluid in arterial ischemic strokes, including apparently idiopathic strokes, may yield essential information on pathophysiology, allowing for optimal therapeutic decisions and prognostic considerations.