It is now recognised that the extrinsic tissue factor pathway is the main trigger to the coagulation system in vivo. Its main inhibitor, tissue factor pathway inhibitor (TFPI), has never been studied in childhood nephrotic syndrome. The aim of the study was to monitor the level of TFPI in childhood nephrotic syndrome. One hundred and thirty-nine nephrotic children were classified into the following groups: group 1 (n=25), in relapse and receiving no treatment; group 2 (n=37), in relapse but receiving steroid treatment; group 3 (n= 45), in early remission and on steroids; group 4 (n=24), in established remission and receiving no steroids; group 5 (n=8), steroid-resistant. The controls (n=84) were healthy and age-matched. There was significant elevation of total TFPI levels in groups 1 and 2 and 3; levels were comparable to those of the healthy controls in group 4. The highest levels of total TFPI were recorded in group 5. Like total TFPI, the levels of the free form of TFPI showed a statistically significant increase in groups 1, 2, 3 and 4, when compared with levels in healthy controls. The highest levels of free TFPI were recorded group 5. We concluded that the elevated levels of both the total and free TFPI in various phases of nephrotic syndrome add another natural anticoagulant mechanism, which will attenuate the hypercoagulability of childhood nephrotic syndrome.