Study design: Case report. Objective: To report an intramedullary germinoma associated with congenital adrenal hyperplasia (CAH). Method: We report a 14-year-old white male with CAH who was found to have an intramedullary germinoma of the conus medullaris. Presentation and evaluation were for progressive urinary incontinence. A brief review of relevant literature was presented. Results: Cases where germinomas have occurred simultaneously with CAH have been identified intracranially, but to our knowledge, this is the first case of CAH and intramedullary spinal cord germinoma occurring simultaneously. Conclusion: Although rare, primary intramedullary germinomas do occur. The association between CAH and spinal cord germinoma should be considered by clinicians. © 2008 International Spinal Cord Society All rights reserved.