Acquired abnormalities of coagulation and fibrinolysis in nephrotic syndrome have been implicated in the pathogenesis of deep-vein and arterial thrombosis. A mutation in the factor V and methylenetetrahydrofolate reductase (MTHFR) gene, the commonest inherited risk factor for venous thrombosis, may contribute to the risk of both arterial and deep-vein thrombosis in patients with nephrotic syndrome. Here, we report on an arterial thrombosis in a young girl with idiopathic membranous glomerulonephritis associated with heterozygous factor V Leiden and homozygous MTHFR C677T mutation. We postulate that screening for factors such as factor V Leiden and MTHFR C677T mutation may be beneficial to patients associated with thromboembolism and idiopathic nephrotic syndrome.