Using fluorescence in situ hybridization, we localized the rat homolog of the von Hippel-Lindau gene (Vhl) to rat chromosome band 4q41.3→q42.1. We also mapped the gene encoding the plasma membrane Ca++-transporting ATPase iso-form 2 (Atp2b2) to the same chromosome subregion. These two genes together with Raf1 appear to be members of a large syntenic gene cluster that maps to human chromosome bands 3p25→p26, mouse chromosome bands 6 C3→E, and rat chromosome bands 4q41→q42. Cytogenetic analysis of NRK 52E cells derived from immortalized normal rat kidney epithelial cells revealed an inverted duplication of the region containing this gene