Thromboembolism is a major complication of nephrotic syndrome (NS). Hypoalbuminemia, loss of anticoagulant proteins, increased procoagulant proteins, hemoconcentration, and platelet activation contribute to a hypercoagulable state. Despite being well-described, the optimal management of thromboembolism in NS remains unclear. Rivaroxaban, a direct factor-Xa inhibitor has recently been shown to be safe and efficacious in treating pediatric venous thromboembolism but has not been well studied in NS. We present an adolescent with steroid-dependent NS, deep vein thrombosis and submassive pulmonary embolism successfully treated with rivaroxaban. We perform a systematic review of the reported safety and efficacy of direct factor-Xa inhibitor in this population.