Primary sclerosing cholangitis (PSC) may have an atypical mode of presentation with recurrent cholangitis and diverticulum-like outpouchings of the hepatic ducts; a high clinical suspicion is required to confirm the diagnosis instead of ascribing cirrhosis to a secondary cause as a result of the biliary stasis propitiated by the biliary cysts. We describe the case of a 63-year old woman with a one-year history of episodes of cholangitis and a persistent elevation of alkaline phosphatase. The endoscopic retrograde cholangiopancreatography and a magnetic resonance cholangiography showed strictures of the intrahepatic biliary tract compatible with PSC and a diverticulum-like outpouching of the right hepatic duct. A liver biopsy revealed cirrhosis. Initial management consisted of antibiotics, a sphincterotomy and the placement of a biliary plastic stent, however, the patient continued to have recurrent cholangitis and finally, the cyst was resected.