This is a rare case of adult primary obstructive megaureter complicated by combined uric acid-oxalate lithiasis of the ureter and renal stones. A 24-year-old male patient presented with frank hematuria on exercise of 4 years duration. The patient had an open surgery in the form of excision of stenotic segment of ureter and left ureteric re-implantation with removal of ureteric and renal stones. Congenital megaureter is a diagnosis that urologists and radiologists need to consider in the setting of isolated distal ureteral dilation, as the diagnosis of adult megaureter may require more involved surgical measures to prevent recurrence of adverse symptoms.