An 8-year-old boy with Wiskott-Aldrich syndrome underwent laparotomy and resection of a stage IE malignant lymphoma of the jejunum. Although preoperative platelet counts were less than 10,000/cu mm, intraoperative bleeding was minimal, and postoperative bleeding from the wound was easily controlled with platelet infusions and local application of epinephrine. Six weeks later, he underwent reoperation for small intestinal obstruction and no residual tumor could be identified. The patient died 4½ months following the initial surgery from an intracerebral hemorrhage. Postmortem examination did not reveal residual lymphoma.