Abstract Background The standard treatment of renovascular hypertension accompanying renal artery stenosis (RAS) consists of angioplasty and administration of antihypertensive medication. Although nephrotic syndrome (NS) has been reported to be associated with RAS, the development of NS after revascularization of RAS is extremely rare. Case presentation A 48-year-old man presented with uncontrolled hypertension and azotemia. The right kidney was atrophic, and RAS of the left kidney was suspected based on a post-captopril DTPA scan. His blood pressure stabilized after renal angioplasty; however, he complained of edema after 1 week. NS developed and was diagnosed as focal segmental glomerulosclerosis (FSGS) based on renal biopsy. He received an angiotensin receptor blocker. Proteinuria resolved after 1 year. Conclusions FSGS rarely develops after angioplasty of renal artery stenosis. This is the first report of successful treatment of this condition using an angiotensin receptor blocker during 1-year follow-up.