Xanthine oxidase deficiency was identified in a 16-year-old girl with acute lymphocytic leukemia (ALL). Despite the enzyme deficiency, the patient tolerated high doses of 6-mercaptopurine (6-MP), a drug which is normally inactivated by the action of xanthine oxidase. This rate clinical situation may be analogous in the practice to that of the patient receiving 6-MP who is also given allopurinol, which inhibits xanthine oxidase activity. The implications of these observations on the determination of appropriate drug dosages for patients receiving these two agents are discussed.