The müllerian system develops from the sixth week of life and it is estimated that the prevalence of congenital abnormalities is approximately 5% [1–3]. These anomalies may be major or minor and may occur in isolation or in conjunction with other congenital malformations or as part of a syndrome. Many patients with simple anomalies will remain asymptomatic and may be diagnosed incidentally while being investigated for other problems or undergoing routine gynecological/obstetric procedures. The others present in a variety of ways depending upon the abnormality present. The commonest means of presentation are primary amenorrhea, obstructed uterus or vagina, dyspareunia, and on investigation for infertility or recurrent miscarriage. Those diagnosed, such as neonates, will usually be patients in whom there are associated major anomalies of the genitourinary and alimentary tracts such as persistent cloacal anomalies and anorectal malformations.