Chronic hemolysis in sickle cell anemia (SCA), results in hyperbilirubinemia, as the water insoluble bilirubin needs to be enzymatically converted into water soluble bilirubin glucoronides, for its elimination through bile by uridine glucuronosyltransferase (UGT) enzyme.1,2 Chronic hyperbilirubinemia, thus over time, can lead to the formation of gall stones (cholelithiasis), a common complication in SCA.1–3 Its onset can be as early as 2 to 4 years, but the prevalence increases progressively with age.4,5. The gene discussed is SLC35A2; the disease is autosomal dominant cerebellar ataxia.