Neurological signs and symptoms are common in Kawasaki disease and most often include aseptic meningitis, encephalopathy, and sensorineural hearing loss. Diagnosis is clinically based on evolving diagnostic criteria. Although stroke in patients with Kawasaki disease has not been methodically studied, several reports exist of hemiparesis in children diagnosed with Kawasaki disease, some of which include angiographically diagnosed vascular lesions. Early attempts to treat Kawasaki disease focused on the use of aspirin, glucocorticoids, and immune modulators such as azathioprine. Review of the current literature suggests that stroke, and specifically asymptomatic brain infarction, may be more common than now recognized, especially in patients with coronary artery abnormalities. Thorough clinical, neuroradiological, and neuropsychological assessments of these patients are indicated to gain a full appreciation of the nervous system involvement and the long-term neurodevelopmental sequelae resulting from this disease.