Marginal Zone Lymphoma (MZL) is a non-Hodgkin lymphoma that develops from B cells, a kind of white blood cell. It is distinguished by the proliferation of aberrant B cells in the marginal zone of lymphoid tissues, which are located close to normal lymphoid follicles. MZL can occur in a variety of organs, including the stomach (gastric MALT lymphoma), salivary glands (salivary gland MALT lymphoma), and spleen (splenic MZL). MZL is distinguished by its slow growth, which frequently causes the disease to stay asymptomatic for an extended period of time. When symptoms do appear, they can differ depending on the site of the lymphoma. Common symptoms include painless enlargement of the lymph nodes, exhaustion, night sweats, and unexplained weight loss. The specific cause of MZL is unknown, but certain risk factors have been discovered. Chronic infections, including Helicobacter pylori in the case of stomach MALT lymphoma, and autoimmune disorders have been linked to an increased risk of developing MZL. MZL is diagnosed using a combination of medical history, physical examination, imaging investigations, and a biopsy. A thorough evaluation is required to identify the severity of the disease and choose a suitable treatment strategy. MZL's indolent nature provides for a wide range of therapeutic options, from a watch-and-wait approach for asymptomatic patients to more aggressive treatments such as chemotherapy and immunotherapy for those with advanced or symptomatic illness. Treatment decisions are made individually, taking into account the patient's overall health, the location and severity of the disease, and the exact subtype of MZL. Rituximab, a monoclonal antibody that targets B cells, is widely utilized in MZL therapy regimens. Other treatments could include chemotherapy, radiation, and, in some situations, targeted medicines. MZL prognosis varies according to numerous criteria, including subtype, stage of diagnosis, and therapy response. Overall, MZL has a better prognosis than other non-Hodgkin lymphomas. Regular follow-up care is critical for tracking illness progression and preventing complications. New therapeutic methods and existing treatments for MZL are still being investigated through research and clinical studies.