Abstract
Introduction Primary synovial sarcoma (SS) of the prostate is the rarest variety of prostate sarcoma. The first documented and confirmed case of SS of the prostate was published by Iwasaki et al in the year 1999; since then, only a few cases of primary SS of the prostate have been published in English literature. Case Report We report a unique case of primary SS in a young patient who presented with acute urinary retention and underwent emergency suprapubic catheterization, and on evaluation was diagnosed with primary SS of the prostate. Patient was managed with radical cystoprostatectomy and resection of the anterior wall of rectum infiltrated by the tumor with bilateral pelvic lymph node dissection and adjuvant chemotherapy. Patient died after 2 months of surgery. Conclusion Primary SS of the prostate is a rare disease and important clinical entity to be included in differential diagnosis of acute urinary retention in young patients. It is associated with high local recurrence and poor prognosis, which warrants multidisciplinary approach of treatment.