We report on a 4-year-old girl with steroid-resistant nephrotic syndrome who presented with life-threatening chylopericardial tamponade with chylothorax secondary to superior vena cava thrombosis. This is the first case report of this complication in a child. We outline the treatment, which included emergency pericardiocentesis and thoracostomy, parentral anticoagulation and dietary modifications. This therapeutic regimen resulted in a good outcome.