Extranodal natural killer/T-cell lymphoma (ENKL) usually occurs in the nose or the nasopharynx, but extranasal and disseminated disease can occur. In this disease, orbital involvement is more commonly seen, but without nasal involvement is rare. A 61-year-old woman was referred with a one-month history of a remarkably enlarging salmon-colored mass arising in the right inner canthus, with redness and painlessness. The motility of the right eye was limited in the medial direction, with external deviation of the eyeball. A magnetic resonance imaging (MRI) scan of the orbits showed a mass of irregular shape located in the right inner canthus, without any sinus involvement. A histopathological examination concluded a diagnosis of primary orbital extranodal natural killer/T-cell lymphoma, nasal type. Her orbital mass significantly reduced to near disappeared after chemotherapy. From the first visit to the present, the survival duration of this patient was more than 1 year. This patient was still alive with a high quality of life and with no systemic metastasis. Extranodal natural killer/T-cell lymphoma, nasal type may primarily arise in the orbit without nasal involvement. Early discovery, early biopsy and diagnosis and early appropriate treatment can successfully control tumors and improve prognosis.