A woman presented with a rapid onset of hypertension, angina pectoris, peripheral vascular disease, renal involvement, and a large liver cyst. Surgical removal of the liver cyst precipitated renal and liver failure and a terminal arrhythmia. At autopsy, there was intimal fibromuscular dysplasia involving the arteries to the heart, liver, kidneys, and intestines and evidence of recent infarction of the intestines, kidney, and liver. This case illustrates that intimal fibromuscular dysplasia (FMD) can be a diffuse and rapidly progressive disease. Some treatments currently being evaluated for preventing restenosis following angioplasty may find use in treating this uncommon disease.