Protein-losing enteropathy (PLE) is a serious complication of the Fontan operation and is associated with pronounced mortality. Medical management of PLE has been only partially successful. A recent report noted dramatic improvement in patients with PLE within 3 weeks of subcutaneous administration of heparin. We report a case of reversal of PLE with resolution of clinical symptoms and normalization of serum albumin, total protein, and fecal alpha1-antitrypsin values after several months of heparin treatment. Our findings substantiate those recently reported but suggest that reversal of PLE may necessitate more than a few weeks of heparin therapy.