Children with Crigler-Najjar syndrome type I are at increased risk for neurologic deficits. Cerebellar symptoms are not prominent and appear in adolescent or adult patients with this syndrome. We report a 2-year-old female with Crigler-Najjar syndrome type I who presented severe cerebellar symptoms revealing bilirubin encephalopathy. The patient improved slowly with the duration of phototherapy. Cerebellar symptoms can be the initial manifestation of kernicterus in children with Crigler-Najjar syndrome type I.