Intramedullary spinal cord metastases (ISCMs) are rare and account for 4-8.5% of central nervous system metastases. Only one case of biopsy-proven ISCM due to prostate cancer has previously been reported. The current study presents an additional unique case of a 74-year-old male who developed symptoms from an intramedullary conus medullaris metastasis as the first manifestation of prostate adenocarcinoma. To the best of our knowledge, this scenario is even more rare and has not previously been reported. The tumor was radically resected, followed by androgen blockade treatment. The patient's neurological deficit significantly improved, with no tumor recurrence during the follow-up period. In addition, the present study provides an overview of the previous literature concerning ISCMs from prostate cancer, and discusses the treatment options.