Ocular adnexal lymphoma (OAL) encompasses a broad spectrum of lymphomas, the majority of which are of the low-grade B-cell non-Hodgkin’s lymphoma (NHL) subtype. By definition, OAL affects structures including the eyelids, conjunctiva, lacrimal apparatus, extraocular muscles, and the orbit. Disease can be limited to a single, localized site, or it can be multifocal. Overlap between ocular adnexal sites is common, and coexisting uveal involvement has been observed. OAL can involve the regional, central, and peripheral lymph nodes as well as distant extranodal sites. Depending upon the lymphoma subtype and extent of disease involvement, there are multiple forms of focal and systemic therapy. The 10-year, disease-specific mortality is approximately 5–10%. Many of the current advances in OAL management have originated from the improvement in our understanding of systemic lymphoma.