The preoperative and even intraoperative differentiation between benign and malignant strictures at the hepatic hilum remains difficult. The aim of this study was to assess clinical, radiologic, intraoperative, and histopathologic findings; surgical treatment; and outcome of patients with Klatskin mimicking benign lesions. Of 49 consecutive patients who were operated on the initial preoperative radiologic diagnosis of hilar adenocarcinoma (Klatskin tumor), 7 (14%) had benign conditions after final histopathologic diagnosis. Pretreatment work-up, therapy, and outcome of these patients were analyzed. Based on preoperative clinical symptoms, imaging assessment, and CA19-9 values, all seven patients were classified as having malignant neoplasms. At laparotomy, the tumors of six patients were judged to be malignant. Five patients underwent hilar resection and concomitant liver resection, and two patients underwent hilar resection alone. There were no operative deaths. The definitive histopathologic examination showed severe cholangitis with extensive periductal fibrosis in all patients. After a median follow-up of 32 months, all patients are well. Clinical presentation and imaging assessment were similar for Klatskin tumors and benign fibrosing disease; therefore, an aggressive resectional approach is justified in any patient with suspicious obstruction of the liver hilum.