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Introduction Portal vein thrombosis (PVT) is defined as the partial or complete occlusion of the portal vein by a thrombus. At the time of diagnosis, PVT may be limited to the main portal trunk or extend to intrahepatic branches and the splanchnic venous axis, including the splenic and superior mesenteric veins. The anatomical extent of thrombosis may be influenced by the underlying etiology, particularly in the presence of thrombophilic conditions.This study aimed to evaluate the association between major thrombophilic disorders and the extent of PVT at diagnosis.Methods We conducted a retrospective study at a university hospital, including patients with imaging-confirmed PVT, whether recent or chronic (with cavernoma). The extent of thrombosis was categorized as:Localized: limited to the main portal trunkExtended: involving intrahepatic portal branches, the spleno-mesenteric trunk, the superior mesenteric vein, and/or the splenic vein.Results A total of 83 patients were included. The mean age was 44.9 years (range: 19–76), with a strong female predominance (77.1%; sex ratio M/F = 0.3).The most frequent underlying cause was natural anticoagulant deficiency (31.3%, n=26), followed by antiphospholipid syndrome (APS) and myeloproliferative neoplasms (MPNs) (13.3% each, n=11), and idiopathic cases (18.1%, n=15). Other causes, mainly inflammatory or infectious, accounted for 24.1% (n=20).The proportion of extended thrombosis varied by etiology:APS: 72.7% (8/11)MPNs: 63.6% (7/11)Anticoagulant deficiencies: 46.2% (12/26)Idiopathic forms: only 20% (3/15)Conclusion In our cohort, the presence of thrombophilic conditions, particularly APS and MPNs, was significantly associated with an extended form of portal vein thrombosis at diagnosis. These findings underline the need for systematic thrombophilia screening in patients with PVT to identify high-risk individuals and optimize early therapeutic interventions and surveillance strategies, especially in cases with potential for extensive thrombotic progression.