Since 1985, cystatin C has been suggested to be a marker of the renal function. Cystatin C is a proteinase inhibitor with a low molecular weight (M(r) = 13359). It is produced at a constant rate in all nucleated cells investigated to date, freely filtered in the renal glomeruli and reabsorbed and catabolised in the proximal tubules. The concentration of serum cystatin C is mainly determined by glomerular filtration, which makes cystatin C an endogenous marker of glomerular filtration rate (GFR). There are few data describing the influence of various factors on the production and elimination of cystatin C. Fully automated assays using particle-enhanced turbidimetry or particle-enhanced nephelometry are available and the assays are precise, rapid and usable in clinical routine practice. Reference intervals have been determined for cystatin C in adults and in children older than one year. It has been suggested that the same reference interval can be used in children older than one year and in adults without gender differences, on the assumption that the same method with the same standardisation is used. Several studies including adults and children with different renal diseases with various kidney function have suggested serum cystatin C to be a better marker of GFR than serum creatinine.