Hilar cholangiocarcinomas, also known as Klatskin tumors, represent a rare subset of biliary tract malignancies. Klatskin tumors are often diagnosed late and are associated with a poor overall prognosis. Here, we report the case of a 51-year-old female, an intellectually disabled and non-communicating individual, who presented for an unrelated medical condition, incidentally detected to have icterus on clinical examination, which on evaluation culminated in the diagnosis of Klatskin tumor. Despite having no attributable symptoms or identifiable risk factors, she exhibited classical biochemical and imaging features consistent with the diagnosis. Though the underlying comorbidities might have masked the presentation in our case, potentially serious liver diseases can remain asymptomatic or have minimal or non-specific symptoms early in their course. Hence, clinical assessments and judicious interpretations of hepatic biochemistry hold the key to prompt diagnosis despite the advancements in modern medicine. Through this case report, we will briefly review the evaluation of cholestasis and hilar cholangiocarcinoma, also known as Klatskin tumor.