This defect culminates in the retention of bilirubin pigment in lysosomes, leading to the liver’s characteristic black appearance.[1–3] Although the prevalence of DJS remains low in the global population, certain ethnic groups, particularly Iranian Jews and Japanese, show a higher increase in carrier rates.[4,5] At the heart of DJS is the AR inheritance of mutations in the ABCC2 gene, a key player in the transport of bilirubin and other organic anions from the liver into bile.[6,7]. The gene discussed is ABCC2; the disease is Dubin-Johnson syndrome.