INTRODUCTION: Klatskin tumor is a malignant neoplasm originating at the hilum of the liver, where the right and left hepatic ducts converge. This tumor is a rare malignancy
given that most Klatskin tumors are unresectable at the time of diagnosis, the prognosis is very poor. CASE PRESENTATION: We present a 58-year-old woman with severe jaundice. Imaging and laboratory findings were consistent with a diagnosis of Klatskin tumor. Given the advanced stage of the tumor, conservative management with percutaneous transhepatic cholangiography (PTC) was implemented to alleviate symptoms. However, due to the severity of the malignancy, The patient died. CLINICAL DISCUSSION: The clinical and radiographic manifestations of Klatskin tumors might overlap with those of inflammatory, infectious, and vascular diseases, making differential diagnosis complex. Despite advancements in imaging techniques and the high accuracy of these modalities, the definitive diagnosis of Klatskin tumors remains very challenging in some cases. CONCLUSION: Considering that Klatskin tumor is a rare and fatal malignancy, early diagnosis during the initial stages of the tumor is associated with a better prognosis and may make the malignancy resectable.