Kernicterus is a severe neurological condition, caused by bilirubin-induced damage in the basal ganglia. The neurological outcome is often poor. In the past decades there seems to have been an increase in the number of reported cases of kernicterus. In order to raise awareness of this condition, we present two patients with kernicterus caused by different pathophysiological mechanisms. In both cases we make suggestions for the improvement of the medical care process. The first patient is a 7-day-old girl with kernicterus due to haemolysis caused by G6PD deficiency. Patient B is a 3-day-old boy with hyperbilirubinaemia based on 0/B blood group incompatibility. Kernicterus resulted in significant disabilities in these children. A proper diagnostic approach and precise treatment of hyperbilirubinaemia are essential to prevent major neurological damage. Awareness of this condition, education of health care professionals and changes in in- and outpatient care are needed to achieve this goal.