Cholangiocarcinoma (CCA) is an uncommon biliary neoplasm that is more frequent in male patients. CCA is categorized into intrahepatic cholangiocarcinoma (iCCA) and extrahepatic cholangiocarcinoma (eCCA) associated with the anatomical origin location. The clinical presentation is non-specific and varies depending on the origin, iCCA is generally asymptomatic until advanced disease is present therefore this neoplasm presents a poor prognosis with a survival rate of two years. We present a case of iCCA with lung metastasis in a 29-year-old male patient with no risk factors for this malignancy.