Purpose. Our objective was to describe characteristic contrast enema findings of fibrosing colonopathy. Materials and methods. We performed barium enemas on 18 children with cystic fibrosis who had received greater than 4500 U of lipase per kg body weight per meal and who had distal intestinal obstruction syndrome unresponsive to medical management. Results. Fifteen patients had abnormal enema results. The most common findings included stricture, loss of haustra, and colonic shortening. Ten patients required surgery, nine underwent colon resection, and one had lysis of adhesions. Pathologists confirmed fibrosing colonopathy in eight of nine speci- mens. Conclusion. Colonic stricture, abnormal haustra, and longitudinal shortening are characteristic barium enema findings of fibrosing colonopathy.