The nephrotic syndrome is a risk factor for venous thromboembolism. This is strikingly apparent in young adults. Portal vein thrombosis as an initial sign of nephrotic syndrome is extremely rare. A 49-year-old male was admitted to our center with complaints of severe abdominal distension, generalized edema, and oliguria for one week. On admission, serum creatinine was 2.1 mg/dl, proteinuria was 3.2 g/ dl, serum albumin was 1.8 g/dl, and platelet count was 13.000/ml. Microscopic hematuria, acute kidney injury, and nephrotic range proteinuria were present. Portal vein thrombosis, massive ascites, and mild splenomegaly were detected by Doppler ultrasound and dynamic magnetic resonance imaging. We present here a case with rapidly progressive glomerulonephritis who developed acute portal vein thrombosis.