A 4-year-old girl with clinical and laboratory signs of Kawasaki disease (KD) was hospitalized and given intravenous immunoglobulin plus aspirin therapy, with rapid defervescence and clinical improvement, and was discharged 48 hours after admission. At the time of her follow-up echocardiography on day 14, orange-brown pigmentation of the nail beds was noticed and confirmed with dermoscopy. No clear association between KD and orange-brown chromonychia has been demonstrated, although reports and case series suggest a possible link between these two entities. We suggest that this particular finding might be encompassed in late (subacute) changes of extremities as part of KD diagnostic criteria.