There is a broad spectrum of benign disorders of the biliary system that resemble hilar cholangiocarcinoma (HCCA) in terms of clinical, pathologic, and imaging findings. No unifying features were found to characterize patients with benign hilar obstruction and distinguish these patients from those with cholangiocarcinoma. Imaging plays a vital role in aiding the differentiation of benign and malignant disease, defining the location and extent of the process, as well as directing biopsy. However, even when lesions at the liver hilum are detected with the highest sensitivity, none of the imaging modalities can reliably characterize and confirm the underlying type of disease. Excessive reliance on cholangiographic or endoscopic biopsy results is dangerous, because tissue sampling is not always diagnostic and a potentially resectable malignancy can be overlooked. Therefore, the preferred treatment option to patients with suspicious hilar lesions should remain resection for presumed malignancy. Local resection with adequate reconstruction excludes a malignant lesion, and provides means of biliary decompression with low mortality and morbidity rate.