Mucosa-associated lymphoid tissue lymphomas (MALT) lymphoma is the most common pathologic type in primary ocular adnexal lymphoma (POAL). Currently, the clinical types and manifestations of primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (POAML) have not been well understood by ophthalmologists, and there is no consensus or guideline for clinical treatment. According to the original location, this paper focuses on the clinical manifestations of early and intermediate-stage POAML, as well as the individualized treatment for each clinical type and lesion range.