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Abstract Background Urethral prolapse (UP) is a rare, benign condition that often goes misdiagnosed and mistreated. It consists of the eversion of the distal urethral mucosa through the outer urethral meatus, leading to vascular obstruction and prolapsed tissue edema, which aggravates the prolapse. Although many review articles were reported on this condition, none of them focused on the conservative approach nor targeted the pediatric population. In this paper, we present successful medical management of UP in a 13-month-old Caucasian female and perform an extensive review of the literature to include articles that reported a conservative approach in patients under 18-years-old. We also suggested a practical algorithm for both diagnosis and management of this condition. Case presentation A 13-month-old Caucasian white female was presented with a urinary tract infection and vaginal bleeding. Genital examination showed a hyperemic donut-shaped mass covering the urinary meatus, of 0.7 cm diameter, with an actively bleeding open center. After confirming the diagnosis of UP with catheterization, a conservative approach was decided; antibiotics associated with topical corticosteroids including betamethasone 1% cream. The first-week outcome showed regression of the prolapsed tissue and the examination after four months showed normal vaginal mucosa with no evidence of relapse. Conclusions 20 studies were retained after PubMed search with a total of 278 female patients aged between 1 month and 15 years old. The main reasons for referral were vaginal bleeding and mass, urinary symptoms followed by sexual abuse. Non-surgical treatment was tried for 162 patients. 53 of them were successfully managed, while 92 reported unsuccessful outcomes. The conservative approach was successful in 19% of all patients and raised to 32.7% in the portion of children selected for the initial conservative approach. Even though this review showed good results with medical treatment in children with UP, additional comparative studies are required to evaluate the effectiveness of conservative treatment in comparison with surgical interventions. In addition, Long-term follow-up is required as recurrence was reported up to 2 years following conservative treatment.