Purpose: Primary sclerosing cholangitis (PSC) is a chronic progressive disease characterized by inflammation, fibrosis and stricturing within the intra and extrahepatic biliary ducts. It is commonly associated with abnormal liver function tests such as alkaline phosphatase, AST, ALT and total bilirubin. The aim of this study is to evaluate patients with PSC with normal liver function tests. Methods: Retrospective cohort study. Results: Of 101 PSC patients, 10 (seven males and three females) had normal LFTs. The mean age was 47.1 years (range 18-69). Five patients had IBD disease (three CD, two UC), four patients did not have IBD, and one patient's IBD status was unknown. One patient had a previous biliary sphincterotomy. Three patients had prior cholecystectomies and one patient had a previous cholecystectomy and choledochoduodenostomy for papillary adenocarcinoma of the gallbladder. On ERCP, five patients had mild to moderate ductal disease, two patients had moderate disease, two patients had moderate to severe disease, and one patient had severe disease. One of the patients was diagnosed with cholangiocarcinoma at time of initial ERCP, and one patient was found to have cholangiocarcinoma 8 months later by biliary brushings on surveillance ERCP. Conclusion: Although PSC patients commonly have abnormal LFTs, there is a subset of PSC patients with normal LFT's. Despite normal labs, these patients can have severe ductal disease and even cholangiocarcinoma. These findings may have significant importance for screening and surveillance for malignancy in patients with PSC. Normal ducts do not appear correlate to quiescent disease.