Wilms tumor (WT) is the most common primary renal tumor in childhood. The Occurrence of WT in patients with growth retardation, mental retardation and central nervous system abnormalities in association with premature chromatid separation (PCS) and mosaic variegated aneuploidy has been previously described in only 10 patients. Here we report the very rare occurrence of WT with two other malignancies, acute myeloid leukemia and medulloblastoma in association with chromosomal instability. This is a novel presentation of Fanconi anemia with this cytogenetic abnormality. Pediatr Blood Cancer 2009;53:208-210. (C) 2009 Wiley-Liss, Inc.