The aim of this study was to add a new case of primary non-Hodgkin's malignant lymphoma of the vulva to the literature and to review the current literature.We searched the PubMed/MEDLINE databases for previous case reports using the key words "non-Hodgkin's malignant lymphoma of the vulva," "vulvar lymphoma," and "primary vulvar non-Hodgkin's lymphoma." We found 29 cases of primary vulvar non-Hodgkin's malignant lymphoma of the vulva reported until 2015. Among them, only 8 cases of diffuse large B-cell lymphoma (DLBCL), classified according to the most recent 2008 WHO classification, were reported.Moreover, only few studies reported the therapeutic management and clinical follow-up of patients affected by this condition.Due to its uncommon presentation, the primary non-Hodgkin's malignant lymphoma of the vulva can be undiagnosed; thus gynecologists, oncologists, and pathologists should be aware of this condition, as a correct diagnosis is essential for an appropriate therapeutic management.