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Bloom syndrome is a rare autosomal recessive disease character ized by growth failure, sun-sensitive facial telang!ectasia, and defective immunity. Cytogenetically, the syndrome is defined by a high frequency of sister chromatid exchanges? Increased risk of cancer has been recognized as a feature of Bloom syndrome since 1965. 2 In children with this syndrome, acute leukemia and lymphoma have been the predominant cancers, wi th solid tumors in older patients. W e now report three unrela ted children with Bloom syndrome in whom Wilms tumor was diagnosed independent ly at three Canad ian centers from 1981 to 1985. The development of Wilms tumor in three pat ients with Bloom syndrome among just more than 100 pat ients worldwide suggests tha t the incidence of this tumor in these pat ients is markedly increased.