Abstract
IMPORTANCE: Bilateral diffuse uveal melanocytic proliferation is a rare sign of several systemic malignant neoplasms. OBSERVATIONS: A patient presenting with uveal melanocytic proliferation underwent a detailed physical examination and extensive imaging. No systemic malignant neoplasm was found. Chorioretinal biopsy was performed, and its immunohistochemical results revealed the presence of primary vitreoretinal lymphoma. CONCLUSIONS AND RELEVANCE: This patient's results suggest that diffuse uveal melanocytic proliferation may be associated not just with systemic malignant disease, but also with primary intraocular tumors, in this case a primary vitreoretinal lymphoma.