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Malignant lymphoma is a tumor of the immune system. It mainly occurs in the lymph node, butit can also originate from extranodal organs such as the gastrointestinal tract, salivary glandand the sinonasal tract. A primary malignant lymphoma in the paranasal sinuses is rare andusually occurs in the maxillary or ethmoid sinus. The sphenoid sinus is a rare primary site forextranodal lymphomas. Moreover, follicular lymphoma involving paranasal sinuses rarely occurs. Recently, we experienced a patient who complained of facial swelling, exophthalmos, reducedvisual acuity, and diplopia for a month, and she was diagnosed with follicular lymphomaof the sphenoid sinus and orbit. Here we report a rare case of follicular lymphoma of the sphenoidsinus and orbit, along with a literature review