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Spontaneous spinal epidural hematoma (SSEH) as an initial presentation of polycythemia vera (PV) is rare. It can present with the features ranging from simple back pain to complete paraplegia depending on the site of the spinal compression. Herein, we describe a case of undiagnosed PV presented as back pain with radiation to the chest wall in a 46-year-old male patient and subsequently diagnosed with spontaneous spinal epidural hemorrhage. To the best of our knowledge, only one case of spinal epidural hematoma-related PV has been reported so far. Emergent decompressive laminectomy was done within 10 h of initial presentation with excellent clinical outcome. Therefore, this condition must be promptly diagnosed and treated with decompression surgery to minimize the risk of neurological complication.