Occipital condyle syndrome (OCS) results from a unilateral occipital pain associated with an ipsilateral paresis of the 12th cranial nerve (hypoglossal), and is typically caused by metastasis of the skull base. OCS diagnosis occurred, in all cases described in the published literature, when metastatic prostate cancer (MPC) was previously known. We present a case of a patient whose initial manifestation of MPC was OCS. The patient was treated with complete hormonal blockade and non-steroidal anti-inflammatory drugs as opposed to locoregional radiotherapy applied in other cases. After 18 month follow-up, the patient had a complete neurological and biochemical response.