Introduction: Leiomyoma is a benign tumor that originates from smooth muscle cells. Despite being the most common benign tumor in women, it is exceptionally uncommon in the urethra, with approximately 150 reported cases. The peak incidence is in the third and fourth decades. The symptoms are location and size related, usually affecting the proximal urethra with a mean diameter of 3.7 cm . The main signs and symptoms are mass sensation in the urethra, dyspareunia, irritative and obstructive urinary tract symptoms, hematuria, and acute urinary retention. Imaging tests such as ultrasound and magnetic resonance imaging (MRI) help diagnose and adequate surgical planning. Complete resection helps avoid recurrence, which may require urethral resection and reconstruction. Methods: A 25-year-old patient with moderate intellectual disability reported vaginal bleeding for 15 days, despite using medroxyprogesterone acetate. Progressive growth of a perineal mass, incomplete bladder emptying sensation and straining were present for one year. She denied hematuria. There was a fixed and painless solid mass of 4 x 3 cm on the anterior vaginal introitus, with minor bleeding at the mobilization. MRI showed a solid and heterogeneous mass, measuring 4.4 x 3.2 x 2.4 cm and no defined cleavage plane with the urethra or anterior vaginal wall. A surgical intervention was proposed. Under general anesthesia, with the patient in the lithotomy position, a U-shape supra-meatal incision was made. Total en-bloc resection of the mass was performed, with an unsuccessful attempt at urethral preservation. The proximal margin was sent to frozen section biopsy with negative result to determine where to stop ressection. Jugal graft urethroplasty was performed using 5-0 polydioxanone. The pubourethral ligament was reconstructed with vycril 2-0. Closure of the vaginal mucosa was made with 2-0 vycril. Results: An anatomopathological study revealed complete resection of leiomyoma. patient was discharged on the first postoperative and had the Folley catheter removed after 3 weeks. At a four-month follow-up, there were no complications or recurrence of urinary symptoms. Conclusions: Urethral leiomyoma is a rare pathology, and there is no established protocol for its treatment. The challenge is resecting the lesion entirely while preserving the structures. It is crucial to reconstruct the urethra and connective tissue support to maintain adequate urinary function. Although it may prolong operation time, jugal graft urethroplasty is a feasible option for large leiomyomas. SOURCE OF Funding: None
