We report a patient with very marked cholangiographic changes of sclerosing cholangitis with normal serum alkaline phosphatase and no other detectable biochemical evidence of cholestasis. He has remained asymptomatic and still has no biochemical evidence of cholestasis despite follow-up over 3 years. This case demonstrates that patients with sclerosing cholangitis may have normal liver function tests including serum alkaline phosphatase, that asymptomatic sclerosing cholangitis may therefore be under diagnosed, and that severe radiological changes of sclerosing cholangitis may not necessarily be associated with a poor prognosis.