A 77-year-old woman was referred for a one-eyed palpebral edema associated with diplopia. An orbit magnetic resonance imaging showed an orbital mass in the superior medial portion of the internal right orbit without any intraorbital involvement. Biopsies demonstrated a nodular lymphoma with mixed follicular grade 1-2 (60%) and large cell components. The tumor mass was treated with a low-dose radiation therapy (4Gy in 2 fractions) with a complete disappearance of diplopia within one week. At 2-year follow-up, patient was in complete remission. To the best of our knowledge, this is the first case of mixed component follicular and large components orbital lymphoma managed by first-intent low-dose radiation therapy.