Acrorenal syndrome is characterized by central longitudinal axis defects of the limbs, ie, split hand and/or foot. Associated renal lesions described so far comprise agenesis, bilateral hypoplasia (originally diagnosed as oligomeganephronia), and duplication abnormalities. The case of a 29-year-old patient with split hand resulting from bilateral aplasia of the third phalanges associated with dysplasia of the third and fourth metacarpals is reported. In addition, the following lesions were noted: hypoplasia of the middle phalanx of the fifth toe, arched palate, pectus excavatum, hypoplastic mammilae, scoliosis, and congenital hip dislocation. The patient presented with hypertension, modest reduction of glomerular filtration, proteinuria, microhematuria, cylindruria, and moderate harmonic hypoplasia of the right kidney on angiography. Glomeruli had no immune deposits on immunohistology. Light microscopy showed widening of the mesangial axis, focal segmental glomerular sclerosis, and renal interstitial fibrosis with occasional foam cells. This case shows that the spectrum of renal abnormalities in the acrorenal syndrome is wider than previously noted.