Congenital defect is an interior or exterior morphologic abnormality of the proper development. The majority of those anomalies are caused by genetic and environmental factors. Therefore it is said that etiology is multifactorial (50% of congenital defects). The clinical presentation of developmental anomalies is variable, from a single defect to variable combination of abnormalities. Urinary tract defects may be due to diverse anomalies, like abnormality of kidney’s number, volume, structure and localizations or renal parenchyma dysfunction. Malformations of genital system may affect the vagina as well as the uterus. The aim of the study was to present the case of delayed recognition of genitourinary multiple defect, that was consisted of doubling of vagina and uterus along with right kidney agenesis. The urinary tract anomalies are commonly accompanied with genital tract disorders. One of the most common developmental defects of female genital system is absence of vagina and uterus that is also called Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome). The cause of MRKH syndrome still remains unknown. The anomaly consists of complete absence of the vagina and the uterus or absence of vagina accompanied with variable abnormality of the uterus. Ovarian function and structure are preserved. When genitourinary anomalies are able to see on medical examination, early diagnosis is possible even directly after delivery. However the anomalies often remain undiagnosed until adolescence. Thus the first sign of the condition is primary amenorrhoea during puberty years. Sexual dysfunctions and infertility come out much later. Often the first signals of the condition are cyclic abdominal pains. Other less common symptoms are voiding disorders, hematuria or persistent, recurrent urinary tract infections which do not undergo routine therapy. Over the past few years the recognition of urinary tract defects has been markedly increased due to high development of diagnostic and therapeutic possibilities, especially a new laparoscopic procedure. Patient’s age, when the genitourinary tract abnormality is recognized, is extremely important. Usually early established diagnosis of congenital malformation is the key of its successful correction. In the future, numbers of late recognitions of genitourinary abnormalities shall constantly decrease, as a result of progress on new imaging possibilities. Summation: 1) The coexistence of congenital malformations of urinary and genital systems has been often observed, for that reason that always should be taking into consideration during therapeutic process. 2) The symptoms of genitourinary defects are usually non specific. 3) As a result of progress in the diagnostic methods, most of the congenital defects including genitourinary abnormalities are recognizable. 4) If the anomaly is early diagnosed, chances for successful chirurgical corrections and psychological therapy will evidently grow.

malformation, genitourinary system, kidney agenesis, imaging methods, children