An opportunity presented itself in the last five years to study both clinically and by postmortem examination two cases of kernikterus. We have also had the privilege of studying the histories and subsequent courses of two children in whom extensive dysfunction of the central nervous system developed following severe neonatal jaundice. It is the purpose of this paper to record these four cases in detail and to review the available literature on the subject. Kernikterus is a term coined by Schmorl<sup>1</sup>in 1903 to designate jaundice of various nuclear masses of the brain, but it was Orth<sup>2</sup>who, in 1875, first described this condition. The structures most commonly affected are the caudate, lenticulate, subthalamic and dentate nuclei, the thalami, the mammillary bodies, the cornua ammonis, the nuclei of the cranial nerves, the olives and even parts of the cerebellar cortex, as well as the anterior and posterior horns