Gastrointestinal diffuse large B-cell lymphoma (GI-DLBCL) is an extremely aggressive form of B-cell non-Hodgkin lymphoma (BNHL) which has complex histological characteristics and manifests a high degree of heterogeneity in terms of clinical, morphological, immunological, and genetic features. GI-DLBCL mainly spreads by infiltrating neighboring lymph nodes, and common gastrointestinal complications (GICS) such as obstruction, perforation, or bleeding, frequently arise during the progression of the disease, posing significant challenges in both diagnosing and treating the condition. Meanwhile, the incidence of GI-DLBCL has been gradually increasing in recent years, and its strong invasiveness makes it prone to being misdiagnosed or completely missed. In clinical practice, over half of the patients diagnosed with the disease are in stage III or stage IV. What makes it worse is that certain patients may not exhibit a favorable response to chemotherapy. All these lead to intricacies in management of this disease. Unfortunately, there is currently no large prospective study or evidence-based medical evidence to provide clear guidance on treatment decisions for this specific type of lymphoma. Neither do physicians have a consensus regarding the optimal approach to address this condition. Recent studies have identified the presence of various prognostic factors that significantly impact survival in GI-DLBCL, which demonstrates the unique particularity of GI-DLBCL, and could help optimize the clinical decision.