A 65-year-old man with sclerosing mesenteritis developed the nephrotic syndrome. Percutaneous renal biopsy revealed classical histologic findings of minimal change nephropathy with a mild interstitial nephritis. Immunomodulation with prednisone led to a rapid and complete remission of the proteinuria but did not alter the course of the underlying sclerosing mesenteritis. The association of lymphomatous and nonlymphomatous neoplasms with minimal change nephropathy has been well-described. Our review of the literature indicates a parallel association of malignant lymphoma with sclerosing mesenteritis and a variety of disorders that constitute a spectrum of disease. The occurrence of this histopathologic form of renal injury and therapeutic response in the setting of a known lymphoreticular disorder suggests a role for a generalized alteration in cell-mediated immunity and not a tumor-induced elaboration of a factor(s) that directly damages the glomerular filtration barrier.