Superficial vein thrombosis (SVT) has regained its place among the venous thromboembolism (VTE) pathology, as a clinical entity, not a benign condition we once neglected. Current evidence demonstrates that SVT can be the initial manifestation of underlying thrombophilia, particularly in cases with recurrent presentation.We present the case of a 25-year-old female who consulted with left lower limb SVT. Initial management included 3-month anticoagulation and compression therapy. After 8 months, A recurrent SVT occurred, so a thrombophilia screening was performed, revealing a  Factor V Leiden homozygous mutation. These findings have indicated a long-term anticoagulation.This case presented SVT as the first and sole manifestation of homozygous Factor V Leiden mutation, highlighting the importance of thrombophilia screening in young patients with recurrent SVT and challenging the traditional view of SVT as a benign condition. Besides, it demonstrates the importance of long-term anticoagulation in a high-risk thrombophilic patient with direct oral anticoagulation as an approved option.This case highlighted the importance of thrombophilia screening within young patients presenting with unexplained or recurrent SVT.  The Homozygous Factor V Leiden mutation represents a severe thrombophilic state requiring aggressive management with Long-term anticoagulation. Future implications suggest updating guidelines for thrombophilia screening in recurrent SVT, individualizing anticoagulation strategies based on thrombophilia severity, and enhancing awareness about SVT as a potential indicator of severe thrombophilia.Activated Protein C Resistance (APC-R), primarily caused by the Factor V Leiden (FVL) mutation, creates a hypercoagulable state. Once considered benign, Superficial Vein Thrombosis (SVT) is now recognized as a significant manifestation of this thrombophilia, especially in recurrent cases. The VTE risk is linked to FVL zygosity, with homozygotes facing a 50-100 fold increased risk. Consequently, guidelines recommend thrombophilia screening for recurrent or idiopathic SVT. For high-risk patients, such as those with homozygous FVL, long-term anticoagulation is justified.SVT can represent the initial clinical manifestation of severe inherited thrombophilia. Appropriate evaluation and long-term management are crucial in high-risk patients, particularly those with homozygous Factor V Leiden mutation. This case underscores the importance of maintaining clinical suspicion for underlying thrombophilia in recurrent SVT and adopting individualized anticoagulation strategies based on thrombotic risk assessment.