Takayasu's arteritis(TA) is a chronic inflammatory and obliterative disease of medium- and large-sized arteries characterized by a strong predilection for the aortic arch and its branches. Renal involvement is usually manifested by renovascular hypertension. Glomerular involvement is rare and largely exists as a mild mesangial proliferative glomerulonephritis, commonly manifesting microscopic hematuria and proteinuria. The association of glomerular disease with TA is of interest since common immunologic mechanisms are proposed for the pathogenesis of both entities. We report a case of TA associated with focal segmental glomerulosclerosis. The patient presented with hypertension(Upper limb; Rt. 200/80mmHg, Lt. 95/60mmHg, Lower limb; Rt. 140/90mmHg, Lt. 110/70mmHg) and nephrotic syndrome(Serum albumin: 2.3g/dL, cholesterol: 454mg/dL, BUN: 18.0mg/dL, creatinine 1.3mg/dL, 24-hour urine protein: 5.17g). Digital substraction angiography showed multiple narrowing and occlusive changes of aortic arch and its branches. Renal biopsy was interpreted as focal segmental glomerulosclerosis. Therapy was instituted with prednisolone.