Orbital lymphoma is a rare malignancy, with diffuse large B-cell lymphoma (DLBCL), a less common but more aggressive subtype. It commonly manifests with progressive proptosis and visual disturbances mimicking conditions such as thyroid eye disease, benign masses, or idiopathic orbital inflammation. A 69-year-old woman presented with right eye proptosis secondary to a progressively enlarging temporal orbital mass. Her condition rapidly deteriorated, with worsening visual acuity, increased proptosis, and elevated intraocular pressure. Imaging revealed an extraconal orbital lesion with widespread systemic involvement, including lytic lesions, hypermetabolic lymph nodes, splenomegaly and skeletal involvement. Fine needle aspiration confirmed DLBCL. She was treated with six cycles of CHOP chemotherapy (Cyclophosphamide, Hydroxydaunorubicin, Oncovin, and Prednisone) and intrathecal methotrexate. Her symptoms resolved, and vision was restored. This case highlights the importance of considering orbital lymphoma as a potential indicator of disseminated disease, and prompt diagnosis and interventions can restore visual functions.