BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic, progressive liver disease, and many patients ultimately require liver transplantation (LT). PSC also confers an increased risk of malignancies, including cholangiocarcinoma (CCA) and colorectal cancer. AIMS: This study aimed to evaluate patient-perceived outcomes and the extent to which these impact health-related quality of life (HRQoL). METHODS: Patients with PSC completed a risk perception questionnaire, the Short Form-36 (SF-36), and the Chronic Liver Disease Questionnaire. Multivariable models were used to determine factors associated with patient-perceived risks of malignancy, LT, and life expectancy, as well as their relationship with HRQoL scores. RESULTS: A total of 95 patients completed the risk perception questionnaire, and 73 returned the remaining instruments. The estimated risks varied widely. Half overestimated their one-year or lifetime CCA risk, while some predicted zero chance. Predicted LT risk was the only outcome concordant with disease severity. Pruritus was associated with higher predicted one-year risks and lower life expectancy. Lifetime CCA and LT risks were associated with the SF-36 physical component score, while perceived life expectancy was strongly associated with mental health domains, including the SF-36 mental component score. CONCLUSIONS: Predicted prognosis varies widely among patients with PSC and is influenced more by symptoms than objective disease severity. The psychological burden of shorter perceived life expectancy impacts mental HRQoL more than the risks of malignancy or LT. These findings highlight an opportunity for improved patient communication regarding these outcomes, as well as the importance of discussing them, as they may impact HRQoL.