ABSTRACT Aim of the Study: To review and suggest treatment approach for rare diseases Leiomyosarcoma of testis. Material and Method: Here, we have reviewed management of Primary Leiomyosarcoma of the testis by search of case reports in PubMed Central, Embase, Cochrane, and Web of Science Central database. We have classified the obtained information as per Oxford Centre for Evidence Based Medicine (OCEBM) level of evidence, and National Comprehensive Cancer Network (NCCN) Categories of Evidence and Consensus. Here, current treatment scenario for sarcoma and its outcome will be discussed for a new case of low grade primary leiomyosarcoma of testis that was confirmed with Immuno-histochemistry after high inguinal orchidectomy, for painless testicular mass. Systematic review, line listing of old reported cases and treatment evidences were analysed by reviewing various international databases. Results: Leiomyosarcoma is a very rare disease, and only few cases have been reported on online databases. It is usually reported among elderly men following irradiation or long-term anabolic steroid use. Intensive search reveals lack of definitive guidelines for management of leiomyosarcoma because available evidence is level V as per OCEBM and level C as per NCCN. Conclusion: Even though early diagnosis and treatment by high inguinal orchidectomy improves survival and decreases risk of recurrence of leiomyosarcoma, there is need of research and definitive chemotherapy guidelines, similar to sarcoma of other site of the...