Most osteochondromas affect the long bones, but rarely originate in the spine. We report an extremely rare case of osteochondroma of the atlas causing obstructive sleep apnea syndrome (OSAS) in a 61-year-old female. The osteochondroma was removed completely using a transoral approach, and the symptoms of OSAS disappeared. A review of the literature regarding osteochondroma confirms the rarity of this lesion and the use of a transoral approach is discussed.