Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder that leads to various long-term complications. However, we are describing an association between this patient’s condition and primary biliary cholangitis (PBC). A 55-year-old male patient presented to our clinic with PBC, demonstrating the importance of how PBC can be clinically asymptomatic and the importance of the criteria given to diagnose the disease. We encourage physicians to periodically check all ADPKD patients to guard against asymptomatic problems that might endanger their health in the future.