Introduction:Nephrotic syndrome (NS) is a clinical disorder characterized by significant proteinuria, hypoalbuminemia, hyperlipidemia, edema, and other complications. Urinary loss of clotting inhibitors, zymogens, and plasminogen; increased hepatic synthesis of fibrinogen and lipoproteins, and hemoconcentration due to fluid loss are some of the factors which predispose NS patients to hypercoagulable states like portal vein thrombosis.Case presentation:In the presenting case report, we described a 21-year-old woman with no history of NS and a hypercoagulable state who presented to our emergency department with severe generalized abdominal pain and lower limb edema. She was subsequently diagnosed with NS complicated with portal vein thrombosis and was admitted to our internal medicine unit. After 2 weeks of treatment, the patient was discharged in good health.Conclusion:Additional evaluation for newly onset NS with venous thrombosis should be needed in the presence of severe abdominal pain and lower limb edema even in a patient without a previous history of NS.