Gastrointestinal lymphomas represent up to 10% ofgastrointestinal malignancies and about one third of non-Hodgkin lymphomas. The most prominent histologies aremucosa-associated lymphoid tissue lymphoma and diffuselarge B-cell lymphoma. However, the gastrointestinaltract can be the site of rarer lymphoma subtypes as aprimary or secondary localization. Due to their rarity andthe multifaceted histology, an endoscopic classificationhas not been validated yet. This review aims to analyzethe endoscopic presentation of rare gastrointestinallymphomas from disease diagnosis to follow-up,according to the involved site and lymphoma subtype.Existing, new and emerging endoscopic technologieshave been examined. In particular, we investigated thediagnostic, prognostic and follow-up endoscopic featuresof T-cell and natural killer lymphomas, lymphomatouspolyposis and mantle cell lymphoma, follicular lymphoma,plasma cell related disease, gastrointestinal lymphomasin immunodeficiency and Hodgkin's lymphoma ofthe gastrointestinal tract. Contrarily to more frequentgastrointestinal lymphomas, data about rare lymphomasare mostly extracted from case series and casereports. Due to the data paucity, a synergism betweengastroenterologists and hematologists is required in order to better manage the disease. Indeed, clinicaland prognostic features are different from nodal andextranodal or the bone marrow (in case of plasma celldisease) counterpart. Therefore, the approach shouldbe based on the knowledge of the peculiar behavior andnatural history of disease.