A ureterocele is a cystic dilatation of the distal ureter with variance in anatomy and presenting symptoms. Ureteroceles can be associated with single or duplex collecting systems. The clinical presentation can be so diverse as to be ranging from totally asymptomatic to overt urinary tract infection. It is multimodal management when it comes to managing a case of ureterocele for optimal outcomes. Literature is still struggling to find best practices guidelines in managing ureteroceles. Basic measures are taken to preserve renal function. Primarily these cases are managed by adopting transurethral incision of the ureterocele which is an easy, minimally invasive and practicable option for dealing patients who have ureteroceles. In this review we tried to explain the importance of diagnosing this rare ailment in time and taking the measures to deal with it in terms of preservation of renal function. New advances and surgical options have also been discussed here in managing such cases.