Purpose Assess the relationship between liver T1 relaxation time and extracellular volume (ECV) fraction and the disease severity of primary sclerosing cholangitis (PSC). Methods This retrospective study included 93 patients with PSC and 66 healthy patients in the control group. T1 relaxation times were measured in the right and left lobe, as well as in the area of stricture. T1(PSC) and ECVPSC were calculated by averaging T1 and ECV of both lobes and stricture site. T1 and ECV were compared between the two groups and according to PSC phenotypes and severity based on Mayo Risk Score (MRS). We also examined the relationship between T1 and ECV with non-invasive measures of fibrosis such as Fibrosis-4 index (FIB-4) and liver stiffness measurement (LSM) by transient elastography. Results Mean liver T1 (774 +/- 111 ms, p < 0.001) and liver ECV (0.40 +/- 0.14, p < 0.05) were significantly higher with both large-duct and small-duct-type PSC which may lack classic imaging findings on MRCP compared to the control group (p < 0.001). T1(PSC) and ECVPSC showed weak-moderate correlation with LSM, FIB-4, and MRS (p < 0.05). Cut-off values of liver T1 to detect patients in low-risk and high-risk MRS groups were 677 ms (AUC: 0.68, sensitivity: 76%, specificity: 53%, p = 0.03) and 743 ms (AUC: 0.83, sensitivity: 79%, specificity: 76%, p < 0.001), respectively. Conclusion T1 relaxation time and ECV fraction can be used for quantitative assessment of disease severity in patients with PSC.