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The risk of development of malignancy is from 2\u201310%, indicating that these individuals have a far greater than chance risk of developing malignancies, in spite of their short life\u2010spans. Tumors are divided into major histologic types: lymphoreticular, 58% of total; leukemias, 17% of total; epithelial. 18% of total; mesenchymal, 3% of total; and nervous system, 4% of total. Data are reported on 14 families in which 12 siblings had the same tumors and identical primary immunodeficiency diseases. The majority of tumors are lymphoreticular or leukemias: these findings are consistent with the hypothesis that individuals with primary immunodeficiency syndromes have intrinsic abnormalities of the lymphoid system which result in increased frequency of malignant transformation and inability to eliminate transformed cells. 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