Congenital nephrotic syndrome (CNS) refers to a disease presenting with massive proteinuria in association
with hypoalbuminemia, hyperlipidemia, and edema at birth or within the first three months of life. In the past, most children
with CNS had extremely poor prognosis and succumbed to various complications, usually within the first 6 months.
Recent advancements in protein supplementation and nutritional support, renal replacement therapy and renal transplantation
in infancy, render these patients to have much better outcomes [1-5]. However, there are still many hurdles in the
management of this disease. Thromboembolism is an uncommon, yet important complication which the healthcare givers
must be aware of. This article reviews the challenges in the management of the thrombotic complications with special
emphasis on the unique characteristics of the newborn hemostasis system and anti-thrombin (AT) depletion in nephrotic
syndrome. Due to the relatively low incidence of CNS in children and scarce information in the literature on the optimal
management of the thromboembolic complications, most of the recommendations are based on the authors’ experience.