Solitary crossed renal ectopia (SCRE) is an exceedingly rare anomaly of the urinary tract. So far, only 34 cases have been reported in the literature. It usually presents after infancy. Most of these cases are diagnosed incidentally while patients are undergoing evaluation for associated genitourinary, cardiovascular, hematological or vertebral abnormalities. We report the first case of SCRE presenting in neonatal age with impaired renal function and vesico-ureteric reflux.