A 9-month-old previously healthy Tamil boy was transferred from a local hospital for further evaluation of fever for 8 days and macroscopic hematuria. The infant had diarrhoea during the initial phase of febrile illness and it subsided spontaneously. He showed macroscopic hematuria and pyuria, but his urine culture was negative. An antibiotic had also been given empirically for a urinary tract infection before he was transferred from the local hospital. However, fever did not respond to the antibiotic. His food intake was significantly reduced. He was crying most of the time. His immunization and development had been normal. On examination, the baby was febrile (103 ˚F), ill, and irritable, and hydration was satisfactory. There was lymphadenopathy in the right cervical region which was 1.5 cm in size and a maculopapular rash was noted all over the body. There was no Bacillus Calmette–Guérin (BCG) reaction. Other systems examinations were unremarkable. Urine microscopy on several occasions revealed hematuria and pyuria. Blood investigations showed high white blood count (24 × 103 /cumm, neutrophils 80%), low hemoglobin (8 g/dL), slightly high platelets (440 × 103/cumm), high C-reactive protein (96 mg/dL), high erythrocyte sedimentation rate (ESR, 80 mm/1st hour), high liver functions [alanine transaminase (ALT) 98 IU/dL, aspartate transaminase (AST) 120 IU/dL, gamma-glutamyl transferase (GGT) 156 IU/dL] and low serum protein (total 5.8 mg/dL, albumin 2.4 mg/dL). Renal function, serum ferritin, and lipid profile were within normal limits. The cerebrospinal fluid analysis revealed normal results. Results of serology for Epstein–Barr virus, cytomegalovirus, influenza, and mycoplasma were within normal limits. Urine, cerebrospinal fluid (CSF), and blood cultures were sterile. The chest x-ray had been normal. Ultrasound abdomen showed mild hepatomegaly with the normal size gall bladder. The echocardiogram (ECHO) showed left coronary artery dilatation (4.75 mm) on day 12 of illness. He was diagnosed to have atypical Kawasaki disease and started on conventional high-dose intravenous immunoglobulin (IVIG) (2 g/kg) and high-dose aspirin (100 mg/kg). He responded very well, within 24 hours of IVIG. Aspirin was continued until ESR became normal and changed to low-dose aspirin subsequently (5 mg/kg). The second ECHO after 2 weeks of treatment showed persistent dilatation of the left coronary artery (3.5 mm). He was discharged after 3 weeks of illness with low dose aspirin for 6 weeks and follow-up in clinic. At 6 weeks, all inflammatory markers had become normal; however, he had 3 mm dilatation in the left coronary artery, and he was recommended for long-term follow-up and low-dose aspirin therapy. He was reviewed monthly with aspirin and repeated ECHO at 1 year showed normal findings. After 2 years of follow-up in tertiary care hospital, he was referred to a local clinic for routine follow-up and immunization.