Low-grade Extranodal Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, a subtype of non-Hodgkin's Lymphoma, involving the kidney is a rare clinical entity. Association of Minimal change disease nephrotic range proteinuria with Hodgkin's lymphoma is well described, however is extremely uncommon with non-Hodgkin's lymphoma. We describe a patient who presented with nephrotic syndrome and a kidney biopsy revealed marginal zone lymphoma and diffuse epithelial foot process effacement. He showed dramatic response to a combination therapy with cyclophosphamide, corticosteroids, and Rituximab.