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The early\u2010 and late\u2010infantile subtypes, which are the most common forms of the disease, are rapidly progressive and lead to early death, whereas the later\u2010onset types are clinically heterogeneous. The only disease\u2010modifying treatment currently available is hematopoietic stem cell transplantation, which is effective only when performed early in the course of the disease. Because most patients with KD are diagnosed too late for treatment, primary care physicians are faced with the challenge of caring for a child with severe neurologic impairment. This Review describes presenting symptoms, diagnosis, and disease manifestations of KD and provides basic guidelines for its management. 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