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The spindle cell rhabdomyosarcoma is a rare variant of the embryonal rhabdomyosarcoma, mostly occurring in childhood. Only a few cases are described in adults. To date, no case of the spindle cell subtype of the prostatic embryonal rhabdomyosarcoma has been published. We report on a 23-year-old man, initially presenting with obstructive micturition problems, perineal pain and night sweat. After diagnosis by transrectal biopsy of the prostate, radiochemotherapy within the CWS 2002 P study was applied: nine cycles of vincristine, doxorubicin, actinomycin D, ifosfamide, and fractionated radiotherapy of the tumor and suspect lymph nodes (final dose 50.4 Gy). The tumor initially shrank, but an early local recurrence arose. Second-line chemotherapy was applied, followed by a salvage radical cytoprostatectomy. The patient died of disseminated disease 14 months after diagnosis.