Background: Cancers of the male urethra constitute less than 1% of all malignant urological tumours, and the occurrence of sarcomas in the urethra is even less frequent. To our knowledge, only one case has been previously described in the English literature., Case Presentation: We report the clinical features, histology, imaging and treatment of urethral leiomyosarcoma in a male patient., Conclusion: The occurrence of sarcoma in the urethra is most unusual, and its appearance as a primary growth in the male urethra is not recorded in the English literature. In conclusion, this case highlights a rare type of primary male urethral malignancy that features a poor prognosis.