The authors report a case of leiomyoma of the female urethra and review the literature concerning this disease. This is a rare tumour that only exceptionally involves the urethra and is therefore difficult to diagnose. Urethral leiomyoma can present in the form of urinary tract infections, detrusor instability, haematuria, dyspareunia, and, more rarely, dilatation of the proximal urinary tract. Treatment is surgical. There is no risk of metastases, but local recurrence can occur in the case of incomplete resection.