INTRODUCTION AND IMPORTANCE: Ectopic ureter is defined as any ureter, single or duplex, that opens in a location other than trigone of bladder. Continuous urine leakage and regular intentional voiding must point to the diagnosis of an ectopic ureter, particularly in females (Singh et al., 2022). Following successful repair of ectopic ureter, the overall long-term continence rate is satisfactory. CASE PRESENTATION: This case is reported to discuss a case of 24 yrs. old woman presenting with a complaint of insensible continuous urinary leak with normal intentional voiding since childhood. Ultrasound and CTU showed left solitary kidney with normal insertion of its ureter; but failed to demonstrate right system. MRI showed Right EU with ectopic dysplastic right kidney. Renal scintigraphy was unavailable at the time of evaluation and IVP was suggestive of NEK. Nephroureterectomy done. Her subsequent follow up was satisfactory. CLINICAL DISCUSSION: Because many people with EU are asymptomatic and the diagnosis is frequently missed, the prevalence of EU is uncertain. Preferred mode of diagnosis is pelvic MRI. Ureteral duplication accounts for 80 % of ectopic ureter occurrences in women (Demir et al., 2015). Ectopic ureters draining a single-system ectopic ureter with dysplastic kidneys, on the other hand, are uncommon, particularly in females (Amenu et al., 2021) Despite this rarity, we have found single system with atrophic kidney. CONCLUSION: This instance suggests to us that in cases of urinary incontinence especially in women, congenital anomalies of the genitourinary tract should be taken into consideration. Surgical management depends on the degree of renal function and location of EU. Either nephroureterectomy or ureteric reimplantation are curative for incontinence.