A 44-year-old man with primary sclerosing cholangitis (PSC), with a mild ulcerative colitis (UC), former smoker, without other significant comorbidities, showed at his last annual abdominal ultrasound (US) newly dilatated intrahepatic bile ducts. Blood tests showed slightly increased cholestasis compared to previous tests (normal bilirubin levels, GGT 155 U/L (previous 67), ALP 176 IU/L (previous 110). He underwent a magnetic resonance cholangiopancreatography (MRCP), which confirmed the presence of dilated intrahepatic bile ducts, while the gadoxetic MR evidenced a 3 cm perihilar biliary stricture, highly suspicious for an extrahepatic cholangiocarcinoma (eCCA). A chest CT scan excluded lung metastases. The patient is asymptomatic with preserved liver function, CA19.9 is 38 IU/mL. Last endoscopy showed neither evidence of active UC lesions nor colorectal cancer lesions.