The incidence of unilateral renal agenesis ascertained at autopsy of human beings averages between one in 600 and one in 1,200 In female the incidence of associated genital anomalies has been conservatively estimated as 50% In most women the genital anomaly consists of some degree of duplication of the uterus cervix and/or vagina and less commonly some form of partial atresia . Embriologically the mullerian ducts develop just lateral to the mesonephric ducts which have been postulated to act as a guide to the growth of the mullerian duct Because the urinary and genital systems are derived from a common embryonic mesoderm it is easy to imagine an early teratogenic influence leading to simultaneous defects in both systems The mesonephric duct abnormality appears responsible for the lack of a normal ureteric dud which results in later renal agenesis The renal agenesis is predominant in the right side but it is not clear why there appears to be a right-sided predominance Uterus didelphys is more commonly observed although a bicornuate or septate uterus is documented in a few instances Recently we have had 3 cases of septate uterus is documented in a few instances agenesis at the Wallace Memorial Baptist Hospital of which one was uterus didelphys and the others were bicornuate uterus and septate uterus.