A 45-year-old man with typical Hutchinson-Gilford progeria syndrome is described. The patient had the characteristic physical findings of this syndrome, such as short stature, "horse-riding" stance, coxa valga, alopecia, micrognathia, craniofacial disproportion, and prominent eyes. He had refractory congestive heart failure due to arteriosclerotic heart disease and hypertension, and he also had arteriosclerosis obliterans. Some immunologic and endocrinologic abnormalities commonly seen in the elderly were present in this patient. On the basis of a review of the literature, this is the first patient with this syndrome who had survived into the fourth decade.