Objective To explore the diagnostic and treating scheme of primary sclerotic cholangitis. Methods 24 cases of primary sclerotic cholangitis identified by radiological and pathological examinations from 1972 to 1998 were analysed retrospectively. According to Thompson, 1 case was classified as type Ⅰ, 5 cases were type Ⅱ, 10 cases were type Ⅲ and 8 cases were type Ⅳ. The operation were as follows,resection of gallbladder plus T tube drainage in 8 cases, plus Roux-en-Y anastomosis of bile duct and jejunum in 12 cases, plus U tube stent and drainage in 4 cases. Results The total mortality rate was 25% (6/24) in 2～18 years follow-up after operation. Conclusion Early diagnosis and operation may resolve the drainage of bile into the jejunum. When serious lesions and worse liver functions exist, liver transplantation should be considered.