The nodal marginal zone lymphoma (NMZL) is a rare indolent lymphoma within the group of marginal zone lymphomas. Although recognized as a distinct entity in the lymphoma classifications since 1994 (REAL and WHO), the differential diagnosis with other lymphomas is still imprecise and clinically requires the exclusion of extranodal or splenic involvement. With regard to treatment, prospective data are limited. Often, the guidelines refer to other indolent lymphomas like follicular lymphoma. Novel drugs were developed parallel to follicular lymphoma or other MZLs, particularly lenalidomide, ibrutinib, or PI3K inhibitors. In the last years, there is an increasing amount of molecular data underlining that NMZL is a separate entity requiring more specific treatments.