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Resumen El tumor de Klatskin es un colangiocarcinoma hiliar que se origina en la confluencia de los conductos biliares. Su causa se relaciona con procesos inflamatorios crónicos, infecciones parasitarias y factores como obesidad, tabaquismo y diabetes. Se manifiesta con ictericia progresiva, coluria, acolia y prurito. El diagnóstico se realiza mediante colangiorresonancia, tomografía y colangiografía endoscópica. El tratamiento ideal es la resección quirúrgica completa o en casos seleccionados, el trasplante hepático tras quimiorradioterapia. El pronóstico es desfavorable, debido a su diagnóstico tardío y alta recurrencia. Abstract The Klatskin tumor is a hiliar cholangiocarcinoma that originates at the junction of the right and left hepatic ducts. Its cause is related to chronic inflammatory processes, parasitic infections, and factors such as obesity, smoking, and diabetes. It presents with progressive jaundice, dark urine, pale stools, and pruritus. Diagnosis is made through magnetic resonance cholangiopancreatography (MRCP), computed tomography, and endoscopic cholangiography. The ideal treatment is complete surgical resectionor, in selected cases, liver transplantation following chemoradiotherapy. The prognosis is poor due to late diagnosis and a high rate of recurrence.