Goals: We aimed to prospectively investigate the full etiologic contributors to portal vein thrombosis. Background: Portal vein thrombosis in the absence of liver disease is a rare cause of portal hypertension with a different clinical course and management strategy. The etiologic distribution of this interesting clinical picture is important as far as diagnostic and management issues are concerned. Study: After the application of exclusion criteria, 59 patients were included in the study who had normal liver functions, normal liver histology and studied the thrombophilia factors of both acquired factors and congenital factors like protein C, protein S, antithrombin levels with the mutations. Results: In all, 23.7% of the patients were found to have acquired thrombophilia factors like myeloproliferative disorders and cyst hydatid disease, whereas 22.1% of the patient population was found to harbor no identifiable cause of thrombophilia, which we termed as idiopathic. One or more causes of thrombophilia were identified in 46 patients. There were 27 patients with protein C deficiency, 18 patients with protein S deficiency. The antithrombin deficiency was found in 17 patients. The factor V Leiden mutation was found in 7 patients. There was I patient with homozygote mutation, whereas the remaining 6 patients were heterozygotes. There were 3 patients with prothrombin mutation who were heterozygote for this mutation. Conclusions: Complete investigation of thrombophilia is crucial to delineate the outline of thrombophilic risk factors to estimate the rethrombosis risk and for further management concerns.