Pathogenesis of PVT is multifactorial. FVL thrombophilia is characterized by poor anticoagulant response to APC and increased risk of venous thromboembolism. This study aimed to determine the prevalence of FVL mutation as a risk factor for PVT in the pediatric age. 40 children with PVT and 20 healthy age and sex matched controls were included in this study. They were subjected to DNA analysis to detect FVL mutation. Prevalence of FVL mutation among the patients (30%) was significantly higher than the control (5%). FVL is frequent in children with PVT and should therefore be assessed.