Purpose To alert ophthalmologists to the possibility of metastatic anterior chamber intraocular non-Hodgkin lymphoma in patients with acquired immunodeficiency syndrome (AIDS). Methods We examined a 19-year-old man with AIDS who had an anterior uveitis with pseudohypopyon and a history of 2 months of malaise. A specimen of the lesion was obtained with a 25-gauge needle for pathologic examination. RESULTS: Systemic exploration showed hepatomegaly. Abdominal computed tomography showed multiple lesions in the liver and spleen. Fine-needle aspiration disclosed a high-grade non-Hodgkin lymphoma. The result of a fine-needle aspiration obtained from a lesion in the anterior chamber was consistent with the same diagnosis. Conclusion An anterior chamber pseudohypopyon with uveitis can be the manifesting sign of a systemic lymphoma in a patient with AIDS and requires a thorough systemic evaluation.