OBJECTIVE: To tell to a case of syndrome of orbital apex the right for low the incidence, distinguishing high morbity and diagnosis. CASE REPORT: AAC, 65 years, masculine, with complaint of sudden chronic headache, frontal, in pressure, of moderate intensity, with right irradiation for hemiface and retro-orbital region to the right, has 15 days. It did not present worsening factors and related improvement to the analgesic use. It evolved with palpebral dyplopia and ptosis to the right, 7 days before being interned Ready way Aid. Requested evaluation of the Service of Otolaryngology after resulted of Computerized Tomography of sinus of the face that bilaterally evidenced density of soft parts in sphenoid sinus. Carried through flexible nasofibrolaryngoscopy, which did not evidence alterations. Introduced venous antibiotic therapy/corticotherapy, nasal topic corticotherapy and nasal hygiene, requested evaluation of the team of the ophthalmology and surgical programming. It after had improvement of the algic picture and extrinsically ocular mobility (vertical mobility of right eye) medicated therapy. In the evaluation of the ophthalmology the patient presented itself without visual perception, however with normal fundoscopy. In the surgical intervention, by previous ethmoidectomy, posterior and bilateral mucous polypoid sphenotomy was visualized aspect mass, material sent for histopathological study. Patient evolved with improvement of pain, however with amaurosis the irreversible right. Currently in pursuing to multidiscipline. CONCLUSION: The fact to present itself with common symptomatology to other neurological, aphtalmologic and otolaryngologic afections becomes the syndrome of orbital apex of difficult diagnosis. The association of the evaluation to multidiscipline, therapeutical diagnosis and precocious, is basic for the favorable evolution of the patient, with improvement mainly, of the visual prognostic.