The clinical, biochemical, radiological, and histological data of a 5-
year-old boy with severe limb deformities and renal failure due to oli
gomeganephronia and renal hypoplasia are reported. This patient repres
ents another example of acrorenal syndrome. This boy has a severe visu
al defect due to pigmentory retinopathy, which has not been reported p
reviously.