A 4-year-old female hospitalized with encephalopathy developed classic signs of Kawasaki disease (KD) on the fifth day after admission, in a report from the Hopital Farhat-Hached, Sousse, Tunisia, and Saint-Luc University Hospital, Brussels, Belgium. Following a 2-day history of fever and progressive loss of consciousness, she had a left-sided hemiparesis, and a rash on the chest. EEG showed slowing, especially on the right side, and CT scan and CSF were normal. C-reactive protein was 4.8 gm/L (normal <0.6). Seizures recurred, coma persisted, the rash became generalized, palms and conjunctivae were injected, extremities swollen, and an echocardiogram revealed bilateral coronary artery aneurysms. KD was then diagnosed and she was treated with iv immunoglobulin and aspirin, with slow recovery of consciousness and motor function. At 3 month follow-up she could stand and walk, and at 12 months she displayed autistic behavior, communication dysfunction, and persistent seizures. MRI showed severe cerebral atrophy.