The U.S. Food and Drug Administration has granted Revlimid Orphan Drug Designation for the treatment of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. Revlimid, known as lenalidomide, is an analog of thalidomide with antiangiogenic and antineoplastic properties. It is developed and manufactured by Celgene, a biotechnology company headquartered in Summit, NJ. It has been approved by the FDA for the treatment of multiple myeloma in combination with dexamethasone and for the treatment of myelodysplastic syndromes associated with a deletion 5q. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue occurs outside the lymph nodes and is a rare form of non-Hodgkin lymphoma with B-cell origin. Due to its uncommon nature, there are no standard treatment guidelines for this type of cancer. Revlimid has shown promising clinical results in patients with relapsed or refractory indolent B-cell lymphoma.