Urogenital sinus is a rare congenital anomaly. We reviewed a case of urogenital sinus anomaly in 6-year-old girl with recurrent urinary tract infection and small bladder capacity who was referred from another hospital in Coimbatore for further management. The external genitalia appeared normal, and an initial sonogram and repeat micturating cystourethrograms did not indicate any urogenital anomalies. She therefore underwent clean intermittent catheterization. Three years later the child underwent investigations like urodynamic study (UDS) and dimercaptosuccinic acid (DMSA) scan and cystoscopy, followed by ureteric implantation and Mitrofanoff procedure. The presentation of urogenital sinus anomaly with recurrent urinary tract infection is rare and the management is complex.