Haemoglobin contains haem. Biosynthesis of heme occurs in mitochondria and cytosol. Glycine and succinyl CoA are the precursors for the synthesis of haem. The porphyria’s are a group of disorders due to abnormalities in the pathway of biosynthesis of haem. Breakdown of haem produces bilirubin. In the liver, bilirubin is made water soluble by conjugation with glucuronic acid and is secreted into the bile. The action of bacterial enzymes on bilirubin in the gut produces urobilinogen and urobilin, which are excreted in the faeces and urine. Hyperbilirubinemia causes jaundice. Jaundice can be classified according to cause as haemolytic (pre-hepatic), hepatocellular (hepatic), and obstructive (post-hepatic). Gilbert’s syndrome, Crigler-Najjar syndrome, Dubin-Johnson syndrome are the inherited hyperbilirubinemias.