The case of a 10.5-year-old boy who had complete right main pulmonary artery thromboembolism complicating steroid-resistant nephrotic syndrome is presented. Despite the massive nature of the lesion clinical symptoms were relatively minor. The thromboembolism was successfully treated with intrapulmonary artery streptokinase. The case highlights the discrepancy that may be found between the clinical and pathological findings in pulmonary thromboembolism in the nephrotic syndrome of childhood.