ABO incompatibility is one form of hemolytic disease of the newborn (HDN). Since the development of antenatal treatment for Rh incompatibility has developed, ABO incompatibility has emerged as the most common cause of HDN. Generally, ABO incompatibility is a benign condition because of the low level of hemolysis that occurs. Infants with ABO incompatibility do require close monitoring and treatment, based on the symptoms. The most common manifestation of ABO incompatibility is hyperbilirubinemia. This hyperbilirubinemia can usually be managed effectively with conventional measures such as phototherapy. However, rare complications such as anemia and, in extreme cases, kernicterus may require blood or exchange transfusion.