We report on a 2.5-year-old boy with severe mental retardation, choreoathetosis, dystonia, muscle rigidity, opisthotonus and severe hearing impairment. He had history of severe hyperbilirubinaemia immediately after birth presumably due to ABO incompatibility. The history and the clinical picture suggested the diagnosis of Kernicterus. The MR imaging examination upon admission revealed bilateral signal intensity increase in the globus pallidum on T2-weighted sequences. Additionally, our patient showed signal intensity changes within the subthalamic nuclei, which is known to be another characteristic area of bilirubin deposition in Kernicterus.