A Median Occipital Condyle is a rare occurrence. We have not found any clinical studies in the literature and myelopathy due to a median condyle has been reported once before. Myelopathy due to anomalies of the craniovertebral junction is rare in neurosurgical practice. We describe a case of a 72-year-old man presenting with progressive myelopathy related to a median occipital condyle located at the anterior foramen magnum region.