Estimated renal function based on serum creatinine can be falsely low and reduced renal function thus go undetected in patients with neuromuscular disorders. Cystatin C, another marker of renal function, could be a better marker of renal function in this patient population since it is not to the same extent afflicted by the patients muscle mass. To investigate the association between measured glomerular filtration rate (mGFR) assessed by iohexolclearence, and estimated glomerular filtration rate (eGFR) with two creatinine-based formulas MDRD (eGFRMDRD) and CKD-EPI (eGFRCKD-EPI) and one cystatin C-based formula (eGFRcysC) in patients with primary neuromuscular disease. Fifty patients (19 M, 31 W, age mean (sd) 46(14) years, BMI 26(6) kg/m 2 ) with DM1, LGMD, FSHD, Duchenne or Becker muscular dystrophy were included. GFR was measured by iohexolclearence and blood samples for analysis of serum creatinine and cystatin C were drawn simultaneously. GFR (mGFR) was mean (sd) 75(16), range 38–117 ml/min/1.73 m 2 (normal > 60) Serum creatinine ranged between 7 and 144 μ mol/l (normal 60–95). Serum cystatin C was 0.98(0.17) in men and 0.88(0.16) mg/l in women, respectively (normal 2 , respectively. Cystatin C-based eGFR was significantly correlated to mGFR ( r = 0.74, p 2 . This overestimation was independent of mGFR in the interval 50–100 ml/min/1.73 m 2 . However creatinine-based eGFR overestimated the kidney function even more. Both creatinine- and cystatin C-based eGFR overestimated renal function in patients with muscular dystrophy. Estimation of renal function using cystatin C-based formulas was somewhat more reliable than creatinine-based formulas, especially in patients with very low muscle mass and extremely low levels of creatinine.