This case report highlights a rare occurrence of urethral prolapse (UP) in a 7-year-old girl diagnosed with cystic fibrosis (CF). The patient displayed a protruding, swollen, and bleeding mass at the urinary orifice, accompanied by chronic constipation and a recent respiratory syncytial virus (RSV) infection. Cystovaginoscopy and circumferential excision were performed, leading to a successful outcome. Since the operation, the patient has been pain-free. The association between CF and UP underscores the need for heightened awareness among clinicians. The report focuses on recognizing and managing UP in pediatric CF patients to provide the best possible care.