Purpose:To present a case of deep orbital dermoid cyst with emphasis on clinical presentation, imaging spectrum, differential diagnosis and management.Case Report:A 28-year-old female was referred to our hospital with chief complaint of drooping of right eyelid and progressive headache. Ocular motility, visual acuity and fundus examination were normal. computed tomography (CT) and magnetic resonance imaging (MRI) revealed a well-defined, intraosseous deep orbital dermoid cyst (5.9 mm × 12.5 mm) located near the apex of right orbit, extending from greater wing of sphenoid into the superior orbital fissure. Due to occulomotor nerve (superior and inferior divisions) compression which passes through the superior orbital fissure, ipsilateral headache and ptosis occurred. Complete surgical excision of cyst was performed using noninvasive extracranial lateral orbitotomy approach. After removal of the cyst, curette and cutting drill were used to thoroughly remove any residual cystic content. Histopathological analysis confirmed the diagnosis. The healing was uneventful postoperatively.Conclusion:CT and MRI are easy, reliable, safe and effective imaging methods for establishing the diagnosis of orbital dermoid cyst. Size, location and manifestations are the most important determinants of the disease management. Complete surgical excision without rupture of the cyst is the treatment of choice.