Objective: To conduct a literature review and present a case study of tubulopapillary cholangiocarcinoma. Material and Method: The clinical record and the clinical, radiological, and pathological characteristics of the tumor were reviewed, along with the scientific literature regarding tubulopapillary carcinoma. Case Report: Patient with progressive jaundice associated with weight loss. Imaging studies show amputation of the distal third of the common bile duct by soft tissue, suggestive of cholangiocarcinoma. Endosono-graphy was performed, yielding “superficial fragments of tubulopapillary adenocarcinoma.” Subsequently, a cephalic duodenopancreatectomy is performed, followed by chemotherapy. Discussion: Cholangiocar-cinoma is a neoplasm of the biliary tract. There are different histological variants, including tubulopapi-llary cholangiocarcinoma. Its diagnosis is based on imaging and pathological studies. The main imaging finding will depend on the pattern of tumor growth: mass-forming, periductal, or intraductal. Among the intraductal types, a biliary carcinoma with tubulopapillary growth and an epithelial pancreatobiliary phenotype has been described. In recent years, they have been of interest due to their better prognosis. Conclusion: Tubulopapillary cholangiocarcinoma is a rare histological variant of cholangiocarcinoma that is associated with a better prognosis than other variants.