A case is reported of a 7 years old boy with Fanconi's Anemia (FA). The aplastic anemia has been treated with androgens. Six years after the confirmation of the diagnosis FA a (pre-)leukemia occurred. Because of the known complications of cytostatics in FA and the bad prognosis, the leukemia has not been treated. The boy died 4 months later. An overview of the literature shows 31 patients with acute non-lymphocytic leukemia in FA, of which the clinical course, chromosomal investigations and therapy are discussed.