Biliary papillomatosis is a rare disease with strong potential for malignant degeneration. Diagnosis is often not easy and most are made intraoperatively. In the present study, five patients with biliary papillomatosis admitted between 1990 and 1997 were reviewed. Their clinical presentation, radiological and biochemical findings were analysed. The aim of the study was to discern a set of characteristic features that would enable an early diagnosis. All of the five patients presented with recurrent episodes of acute cholangitis and epigastric pain with raised serum alkaline phosphatase. Imaging modalities including ultrasound, CT, endoscopic retrograde cholangiopancreatogram, MRI and magnetic resonance cholangiopancreatogram were reviewed. Salient imaging features included a dilated biliary tree with multiple ill-defined and fuzzy filling defects or endoluminal frond-like mass lesions. In conclusion, biliary papillomatosis is a rare but important cause of biliary obstruction with relapsing cholangitis and obstructive jaundice. With a healthy index of suspicion, the diagnosis can be reached when the above features are available.