The diagnosis of IgG4-SC requires a high index of suspicion and understanding of clinical features is the first step of suspicion. Early recognition of IgG4-SC may lead to proper management with corticosteroids and can eventually prevent organ failure that could possibly result from a delayed diagnosis. On the contrary, a misdiagnosis of IgG4-SC as other types of sclerosing cholangitis or cholangiocarcinoma may delay the optimal treatment or result in unnecessary operation. However, the diagnosis can be challenging in some patients because of the various presentations of the disease and similar presentations of other disorders causing biliary strictures. Therefore, IgG4-SC should be considered in the differential diagnosis in all patients with unexplained biliary strictures.