Collapsing focal segmental glomerulosclerosis can be idiopathic but has been associated with human immunodeficiency virus infection as well as lymphoproliferative, myeloproliferative, and autoimmune disorders. It has a more rapid progression to end-stage renal failure. Treatment of this condition is poorly defined with no clear evidence of response to any treatment regimens. We report a case of collapsing focal segmental glomerulosclerosis in association with multiple myeloma that underwent partial remission following treatment for the underlying disease.