A review of MALT type lymphoma of ocular adnexa is proposed.
Over the last decades, we have assisted to an increase in the incidence of primary ocular adnexa lymphomas (POALs).
The ocular tissue biopsy, before the initiation of the appropriate treatment, is mandatory and necessary for a correct diagnosis of POALs.
Among the POALs, the most common is the primary extra nodal lymphoma of MALT-type (POAML).
The clinical manifestations are heterogeneous and its management requires a multidisciplinary approach involving ophthalmologists, hematologists and radiotherapists.