Venous thrombosis is a common complication of nephrotic syndrome, and most often occurs in the renal veins or in the veins of the lower limbs. Portal vein thrombosis is quite uncommon in patients with nephrotic syndrome, and usually only occurs during treatment or recurrence of the condition. In this Case Study, Sun and Xu present a 52-year-old man in whom portal vein thrombosis was the first sign of nephrotic syndrome.