The authors report on a 9-year-old child who underwent surgery to remove a tumor of the hepatic hilum with preoperative radiographic studies suggestive of malignancy, but whose surgical specimens showed a peculiar fibrosing disease. The lesion was localized to the bifurcation of the hepatic duct, where the bile duct wall and the surrounding tissue was markedly fibrotic. No malignant cells or epithelial destruction were seen. The patient's postoperative course was uneventful, and he is without any sign of recurrence 2 years after surgery. Because the histological features of this case do not correspond to any established disease, including primary sclerosing cholangitis, the authors believe it represents a new entity, segmental pericholangial fibrosis. Local resection resulted in a good outcome. A review of the literature disclosed a few similar cases with a benign clinical course.