Cases of secondary sclerosing cholangitis associated with non- Hodgkin`s lymphoma (NHL) are uncommonly reported, and the majority is thought to be associated with a disturbance in the immune system. While the mechanisms of primary and secondary sclerosing cholangitis remain as yet unknown, steroid therapy is recommended for the treatment of the autoimmune variant given the theory of immunologic dysfunction. Here, we present a case of sclerosing cholangitis associated with NHL, who had a successful treatment response.