Purpose: To report the clinical, histopathological and molecular biological fi ndings of a primary extranodal marginal zone B-cell lymphoma (EMZL) of the uvea . Methods: Conventional histology, immunohis tochemistry and polymerase chain re action for immunoglobulin heavy chain gene rearrangement (IgH-PCR) and GeneScan analysis were performed on a chorioretinal biopsy. Results: Histological examin ation of the chorioretinal biopsy demonstrated a dense infiltrate of small centr ocyte-like cells, plasmacytoid tumour cells and occasional blasts. The tumour c ellswere positive for CD20, showed monotypical expression for Ig-kappa and IgM, and a growth fraction of 10%. Clonality analysis using IgH-PCR disclosed a mo noclonal B-cell population. Following localised irradiation of 35 Gy, a complet e remission was achieved. Conclusion: This report describes a rare uveal EMZL di agnosed following investigation of a chorioretinal biopsy. Despite its rarity, o phthalmic pathologists should consider the diagnosis of a primary uveal EMZL whe n reviewing chorioretinal biopsies. Purpose: To report the clinical, histopathological and molecular biological fi ndings of a primary extranodal marginal zone B-cell lymphoma (EMZL) of the uvea. Methods: Conventional histology, immunohis themochemistry and polymerase chain re action for immunoglobulin heavy chain gene rearrangement (IgH Results: Histological examin of the chorioretinal biopsy demonstrated a dense infiltrate of small centr ocyte-like cells, plasmacytoid tumor cells and occasional blasts. The tumor c ellswere positive for CD20, showed monotypical expression for Ig-kappa and IgM, and a growth fraction of 10%. Clonality analysis using IgH-PCR released a mo noclonal B-cell population. Following localized irradiation of 35 Gy, a complet e remission was achieved. Conclusion: This report describes a rare uveal EMZL di agnosed following investigation of a chorioretinal biopsy. Despite its rarity, o phthalmic pathologists should consider the diagnosis of a primary uveal EMZL whe n reviewing chorioretinal biopsies.