Osteochondromas are the most common primary benign bone tumors which can be either solitary or multiple in the form of hereditary multiple exostosis (HME). Osteochondromas are located frequently in the long bones and rarely involve the spine. Cervical spine remains the most common site for spinal osteochondroma. However, majority of the cases are neurologically asymptomatic as most of them are slow growing with growth directed outside the spinal canal. In this case report, we describe a rare case of solitary osteochondroma arising from C1 vertebra (atlas) resulting in serious neurological complications, ultimately necessitating surgical intervention.