To assess the characteristics of inflammatory bowel disease and disease prognosis among children with elevated gamma glutamyltransferase (GGT) and primary sclerosing cholangitis (PSC)-ulcerative colitis (UC). Our longitudinal, population-based cohort comprised all children and young adults diagnosed with UC in the Canadian province of Manitoba between 2011 and 2018. Diagnosis of PSC was confirmed based on a combination of cholestatic biochemical markers and cholangiographic features. The Fisher exact test with Bonferroni correction was used to examine the relationship between categorical variables. We enrolled 95 children with UC/Inflammatory bowel disease-unclassified with a median age at diagnosis of 14years (IQR: 10.4-15.9years) and 1399 person-years follow-up. Among them, 9 children developed PSC-UC, with an incidence rate of 6.43 new cases per 1000 person-years. In this cohort, 8 (72.7%) of 11 children with high baseline serum GGT levels developed PSC-UC in comparison with 1 (1.2%) of 84 children with normal serum GGT levels at baseline (P<.001). All children with high serum GGT levels at diagnosis had pancolitis in comparison with 63.9% of children with normal serum GGT levels (P=.01). Children with high serum GGT levels were more likely to be perinuclear neutrophil antibodies-positive than those with normal levels (90.9% vs 52.0%, P=.01). Our findings indicated that pediatric patients with UC and with even mild elevations of serum GGT levels, especially at baseline, might be predisposed to develop PSC.