Jaundice is yellow discoloration of the skin and mucous membranes due to hyperbilirubinemia. It is especially common in the neonatal period, more than 60% of term and 80% of preterm infants develop jaundice in the first week of life. Most of these cases occur as a reflection of physiological processes in the neonatal period. But jaundice can be a reflection of pathological processes, and then it is caused by inadequate bilirubin metabolism due to increased bilirubin production, insufficient intake of unconjugated bilirubin into liver cells, bilirubin conjugation disorder, decreased excretion or increased enterohepatic circulation. Jaundice usually begins on the face and spreads cephacaudally. It is important to notice it early because untreated total serum bilirubin levels greater than 30 mg / dL (500 μmol / L) are associated with the neurotoxicity of bilirubin, which can seriously endanger the newborn. When evaluating jaundice, it is crucial to identify whether the cause is unconjugated or conjugated hyperbilirubinemia because there are differences in therapeutic approach. While unconjugated jaundice is most often treated with phototherapy and exanguinotransfusion, and possibly with the use of pharmacotherapy, conjugated jaundice is mostly treated surgically, and optimal nutrition is also important. In this review paper, various causes of unconjugated and conjugated hyperbilirubinemia are listed and described. Also, the importance of early detection of neonates at increased risk of developing significant hyperbilirubinemia, careful monitoring, treatment and follow-up of neonates with neonatal jaundice is emphasized.