Kawasaki disease is an acute multisystemic vasculitis. We report the case of a 10-month-old boy received for a fever of 38.7° Celsius persisting on usual antipyretics for 10 days and bilateral conjunctivitis. The patient had bilateral serous rhinorrhea, polymorphic rash with diffuse perineal erythema, bilateral angular cheilitis, erythematous throat, plus perioral, periorbital and trunk desquamative lesions. There was microcytic hypochromic anemia at 9.5g/dL, thrombocytosis at 760x103/mm3,

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... leukocytosis at 28.27x103/mm3, positive C-reactive protein at 58 mg/L and an elevated sedimentation rate at 88 mm at the second hour. Aspartate amino transferase acid (AST) was elevated to 30 IU/L and alanine amino transferase acid (ALT) to 45 IU/L. Gamma glutamate transferase (γ-GT) was elevated at 488mg/dl. Cytobacteriological examination of the urine indicated aseptic pyuria. Cardiac Doppler ultrasonography showed low-grade pericarditis without coronary involvement. In the presence of prolonged fever > 5 days: bilateral conjunctivitis, oropharyngeal involvement, polymorphic rash, CRP >30mg/dL, VS >40mm/h, thrombocytosis, elevated transaminases and gamma GT, aseptic pyuria and pericarditis, we retained the diagnosis of Kawasaki disease in its incomplete form. The patient had been treated with acetylsalicylic acid 50mg/kg/24h. The evolution was favorable with stable apyrexia, regression of mucocutaneous lesions and normalization of cardiac ultrasound.