Objective:To retrospectively review a cohort of adult patients with single ectopic ureter, a rare congenital abnormality where one ureter terminates in an abnormal position outside of the trigone of the bladder. Methods:We identified 5 patients diagnosed with a single ectopic ureter over a single surgeon’s career over 35 years and 2 institutions. Clinical symptoms, medical history, interventions, available radiologic imaging, and urodynamic features were analyzed. Results:Median age at diagnosis was 53 years (range 18–85 years). The majority were male (80%). All patients were noted to have a solitary functioning kidney. A diminutive contralateral kidney and ureter was found in 1 (20%) patient. All patients were symptomatic, with the most common presenting symptoms being chronic dysuria (60%) and chronic prostatitis (40%). Physical examination findings were identified in 3 (60%) patients. Interventions were principally dependent upon the type and severity of each patient’s symptoms, and included observation, drainage of ectopic structures, vaginal marsupialization, and laparoscopic nephroureterectomy. The 3 (60%) patients that underwent intervention remained symptom free and the 2 (40%) patients that decided on observation were minimally symptomatic. Conclusions:Single ectopic ureter is a complex embryological abnormality that can result in a wide array of anatomic variations and clinical findings in both men and women. Given the rarity of this entity in adult patients, a high index of suspicion must be held by the clinician for this diagnosis. Ultimately, treatment options for these patients will be dependent upon symptom severity, anatomic presentation, and patient quality of life.