To report an unusual case of primary paranasal sinus lymphoma associated with intraocular secondary uveal involvement. Retrospective case report emphasizing the histopathologic diagnosis as well as imaging studies, and review of the pertinent literature. The diagnosis of ophthalmologic lymphoma can be difficult due to the infrequency of the disease, the diverse presentation, and the need for biopsy for definitive diagnosis. Prior clinical history and systemic testing may be important confirmations in diagnosing such cases.