The mammalian polycystic kidney disease (PKD) gene family comprises eight members whose role in cell physiology is still poorly understood. Two of the founding members of the PKD family, PKD1 and PKD2, are responsible for the majority of cases of autosomal dominant polycystic kidney disease. The present study focuses on a PKD1 homologue, mouse polycystic kidney disease and receptor for egg jelly (PKDREJ) and its putative role in mammalian fertilization. To examine PKDREJ tissue distribution multiple‐tissue Northern blot analysis was performed. We observed that PKDREJ expression is confined to mouse testis. A PKDREJ transcript was detected in spermatogenic cells by in situ hybridization with mouse testicular tissue. Upon heterologous expression PKDREJ was retained in intracellular membrane compartments and unlike PKD1 did not undergo cleavage in the G‐protein‐coupled receptor proteolytic site domain (GPS). Immunocytochemical experiments on isolated epididymal mouse spermatozoa using PKDREJ‐specific polyclonal antibodies revealed that the protein is localized in the acrosomal region and on the inner aspect of the falciform‐shaped head. To precisely characterize PKDREJ expression in the acrosomal region, transmission electron microscopy was performed. Immunogold labeling was only visible at the plasma membrane of the mouse sperm head. Collectively, these data suggest PKDREJ to be a sperm plasma membrane protein presumably contributing to transmembrane signaling in mammalian spermatozoa. Mol. Reprod. Dev. © 2005 Wiley‐Liss, Inc.