Ureteral endometriosis accounts for about 5 % of urologic endometriosis, with a peak incidence in the age group of 30-35 years, usually with unilateral onset. Because of its insidious onset, often combined with asymptomatic loss of renal function, and the ineffectiveness of medications, surgery is the gold standard of treatment.Thus, here we report a case of ureteral endometriosis with severe hydronephrosis to raise awareness of this condition.