We have presented an unusually swift progression of progressive systemic sclerosis (PSS), with death from cardiogenic shock in a 22-year-old woman who had severe hypertension and acute renal insufficiency. She arrived at our hospital with pericardial tamponade and shock. Despite initial improvement after pericardiocentesis, the patient's condition soon deteriorated and she died of cardiogenic shock. PSS was diagnosed at autopsy. Although the course of PSS is frequently indolent, it may also be fulminant, leading to death before diagnosis is determined.