Immune checkpoint inhibitors (ICIs) have been indicated for several cancers; however, these treatments may cause immune-related adverse events (irAE). 1 According to our previous study, immunerelated liver injury (liver-irAE) is a relatively rare adverse event, with incidence rates of 2.7% and 5.3% for grades 2 and ≥3 liver injury, respectively. Additionally, over 50% of the patients with liver-irAE showed a cholestatic or mixed, but not hepatocellular, pattern. 2 Recently, a few studies have suggested that immune-related sclerosing cholangitis (irSC) is caused by ICIs. 3,4 The irSC is more widely recognized by clinicians, and is diagnosed in approximately 7% of all liver-irAEs of ≥grade 3. 2 The irSC shows predominant elevation of γ-glutamyl transpeptidase (γ-GT) and alkaline phosphatase (ALP) than liver transaminases with extrahepatic biliary duct dilatation in the absence of obstruction on imaging. Moreover, the effects of steroid therapy, which is the main treatment for irAEs, 5,6 are known to be limited in irSC. 7 However, there are only a few studies on irSC that fol-