Ureterocele is defined as a congenital abnormality characterized by cystic dilatation of the distal intravesical portion of the ureter. It has an incidence of about 1 in 500 to 1 in 4000 individuals and shows a female preponderance and a ratio of about 4-6:1(Female: Male) with prevalence among the Caucasians. This is a forty-year-old male who was referred to the radiology department for intravenous urography (IVU) following history of recurrent right loin pain since childhood and also right hydroureteronephrosis following several abdominopelvic ultrasounds scans. The IVU showed bulbous dilatation of the distal intravesical portion of the right ureter with surrounding circumferential filling defect within the contrast opacified bladder giving the so-called “Cobra-head” appearance of intravesical/orthotopic ureterocele. Dilatation of the right ureter and collecting systems were also demonstrated. The complimentary ultrasonography showed a cyst within a cyst appearance on the right conforming to intravesical ureterocele, right hydroureteronephrosis was also demonstrated. The patient had courses of antibiotics with subsequent Transurethral resection in the referring health care facility. We report a case of intravesical ureterocele in a 40-year-old man with recurrent loin pain and urinary tract symptoms since childhood.