A 37-year-old man diagnosed with minimal change nephrotic syndrome at the age of 17, was admitted to hospital with leg pain. He had relapsed nephrotic syndrome and thromboembolism of the right femoral and middle cerebral arteries. He was treated with steroid pulse therapy and thrombectomy. Right lower extremity necrosis developed and a below-knee amputation was performed. His nephrotic syndrome and the amputated lower extremity necrosis subsequently improved. Arterial thrombosis is a rare complication of nephrotic syndrome; however, it is a severe complication. Prophylactic anticoagulation should be considered in patients with an increased risk of thrombosis.