Abstract. Primary non-Hodgkin lymphoma (PNHL) of the sphenoid sinus is a rare neoplasm that poses a diagnostic challenge to clinicians. The proximity of the optical nerve and canal to the sphenoid sinus is accountable for the high incidence of visual disturbance in PNHL of the sphenoid sinus. In particular, patients whose radiologic diagnosis reveals bone destruction in the lateral wall involved with optical-nerve-canals or cavernous sinus present a high risk of rapidly developing unilateral blindness. The present study reports 2 rare cases of PNHL of the sphenoid sinus. Sudden sight loss may follow minimally invasive biopsy. In such cases, the measures that must be taken for the prevention of permanent sight loss are limited in the absence of the final pathologic diagnosis.