Wilms' tumor (WT) is the most common abdominal cancer of children. We obtained tumor specimens from 11 patients with WT. Cytogenetic analysis was successful in eight patients. Four showed consistent abnormalities, two had consistent deletions, and the other two had hyperdiploid chromosome numbers with structural aberrations as well. We report the clinical status, histology, and cytogenetics of these eight patients. In addition, we have reviewed the literature on chromosome abnormalities in patients with WT in an attempt to correlate specific abnormalities with disease stage, histology, and clinical outcome. This review includes data from 170 tumors, including tumors from our eight patients. Hyperdiploidy was the most common abnormality, occurring in all stages except stage V. However, only five stage V tumors have been reported. A normal karyotype was found in only 17% of the tumors, but these may be underreported in the literature. Pseudodiploidy occurred in 28% of WTs, and all showed favorable histology. Correlation of chromosome abnormalities with clinical outcome was difficult because of the relatively few cases (35%) for which this information was provided in the literature.