Rotor syndrome is a rare, benign familial disorder characterized by chronic 
fluctuating, nonhemolytic and predominantly conjugated hyperbilirubinemia with 
normal hepatic histology. In contrast to Dubin-Johnson syndrome, there is no 
liver pigmentation in Rotor syndrome. A 36-year-old man was admitted due to 
asymptomatic persistent jaundice. His siblings had jaundice with direct 
hyperbilirubinemia. Physical examination revealed icteric sclerae without 
hepatosplenomegaly. Laboratory findings showed increased serum bilirubin with 
direct bilirubinemia. Hepatic uptake and storage capacity of indocyanine green 
was markedly reduced, while excretion into bile was slightly suppressed. 
Markedly decreased hepatic uptake and poor visualization of the gallbladder and 
biliary tract were shown in 99mTc-DISIDA scan. Histology of the liver showed 
mild steatosis without pigmentation. We report a case with the review of 
literature.