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Immunoglobulin G4 sclerosing cholangitis (IgG4-SC) is a biliary tract manifestation of IgG4-related systemic disease. We investigated the demographics, clinical features at presentation, treatment response, and outcomes of IgG4-SC using data from a large-scale survey in Japan.We performed a retrospective cohort study of 527 patients with IgG4-SC (436 female; median age, 66.2 years; range, 23–89 years) in Japan from 2000 to 2015. Data on patient demographics, presentation, treatment response, and outcomes were collected from questionnaires given to patients at 211 referral centers in Japan in 2015. Patients were diagnosed with IgG4-SC based on the clinical diagnostic criteria established by the Japanese Biliary Association in 2012. Patients were followed for a median of 4.1 ± 3.1 years. Survival was assessed using the Kaplan-Meier curve and log-rank test.Symptoms at presentation included jaundice (35% of patients) and pruritus (13% of patients); 28% of patients were asymptomatic. It was extremely rare for patients with IgG4-SC to present with symptoms of decompensated cirrhosis. At presentation, serum levels of IgG4 were increased (>135 mg/dL) in 84% of patients. Response to prednisolone was excellent in 90% of patients. No patients received liver transplants, and cholangiocarcinoma was found in only 4 patients (0.7%). Restenosis of bile ducts was observed in 19% of patients but did not affect overall survival.In a retrospective cohort study of 527 patients with IgG4-SC in Japan, we found the disease to be benign. Most patients (90%) respond to treatment with prednisolone and few develop decompensated cirrhosis or cholangiocarcinoma.