BACKGROUND AND AIMS: Primary sclerosing cholangitis (PSC) is a rare, cholestatic liver condition that has a significant association with inflammatory bowel disease (IBD) and impaired health-related quality of life (HRQOL). We aimed to characterise and compare validated PSC and IBD patient-reported outcomes (PRO) and determine if PRO correlated with the severity of liver disease. METHODS: We conducted a multicentre, prospective cohort study in Australia enrolling adult patients with PSC from December 2019 to August 2023. Consecutive patients completed a baseline validated PSC PRO questionnaire and a short IBD questionnaire (SIBDQ) if they had concomitant IBD. Linear regression was used to explore factors associated with poor HRQOL and the association between each HRQOL domain and the severity of liver disease. RESULTS: A total of 55 PSC patients were included. Most patients were male (32/55, 58%) with a median age of 44 years and a median 7 years of follow-up from PSC diagnosis. Mental and physical fatigue were the most prominent PSC symptoms. All HRQOL domain scores except PSC symptoms were significantly associated with liver disease severity. Decompensated cirrhosis and a history of cholangitis were associated with reduced HRQOL in various domains, whereas older age was protective against emotional distress (p = 0.01). IBD bowel symptoms and emotional impact had a positive correlation with PSC symptoms and emotional impact within the same patient. CONCLUSION: This is the first Australian study describing PSC and IBD PRO in a well-characterised prospective cohort. We have demonstrated that PSC-related PRO is significantly associated with liver disease severity. Further large-scale studies are required to further interrogate these findings.