a 57-year - old woman presented with a three - day history of photophobia and diplopia in the left eye . she was diagnosed with histologically confirmed nasal nktl of the right nasal cavity one month prior . the snellen visual acuity of the affected eye with correction was 1.0 / 0.63 , and the intraocular pressure in the right and left eyes was 17 and 18 mmhg , respectively . the patient had mild left eye conjunctival injection , ptosis , and swelling of the eyelid . opthalmoplegia , vitreous opacity , and an iris mass developed in the left eye ( fig . thirty - five percent of the cells obtained from the aspiration were morphologically atypical lymphocytes . skin biopsy confirmed neoplastic infiltration of small- to medium - sized pleomorphic lymphocytes with irregular nuclei , inconspicuous nucleoli , and scant cytoplasm ( fig . immunohistochemical staining was positive for cd56 ( fig . 2c ) and cd3 ( fig . the patient was diagnosed with nasal nktl with ocular involvement . after receiving radiotherapy at 900 cgy , her visual acuity improved and her anterior uveitis , vitreous opacity , and iris mass resolved ( fig . 3a and 3b ) . however , the patient 's condition continued to deteriorate despite radiotherapy and chemotherapy , and her ptosis and ophthalmoplegia gradually progressed . extranodal nktl , including nasal nktl , previously known as lethal midline granuloma , is a definitive diagnostic entity according to the world health organization lymphoma classification . however , histopathologically identical tumors may be identified at other extranodal sites , including the skin , muscle , gastrointestinal tract , liver , kidney , and retroperitoneal space . did report a case of histologically confirmed nasal nktl with intraocular involvement , however , in most cases intraocular involvement has not been confirmed histologically . the presence of intraocular involvement raises the possibility of leptomeningeal or central nervous system dissemination , and prompts consideration for external - beam radiotherapy to the eye and orbit in addition to systemic chemotherapy . nktl involving the ocular adnexa is generally a rapidly progressing disease , with a short survival from time of diagnosis , despite standard therapy . this was true in the current case as well ; the patient died three months after diagnosis . since nktl with ocular and orbital involvement is very rare and is characterized by rapid disease progression and a poor prognosis , nktl should be considered in the differential diagnosis of uveitis or orbital pseudotumor refractory to therapy .