Congenital hypoplasia of the adrenal glands usually is associated with certain malformations of the central nervous system, mainly in anencephalic monsters1,2or in combination with other anomalies.3,4Much less common is an isolated congenital adrenal hypoplasia. Including his own two cases, Mosier5found only five known cases.6-8Two of these, however, also had marked hypoplasia of the pituitary gland. The patient to be described in this case report, a boy who died when he was 33 days old, showed the characteristic clinical features of dysadrenocorticism, and autopsy revealed extreme hypoplasia of the adrenal glands. Report of Case The mother was a healthy 22-year-old Rh-positive WR-negative primigravida and primipara. The pregnancy was uneventful; labor was spontaneous, and delivery was uncomplicated. The presentation was cephalic. The full-term baby cried at once, was not cyanotic, and showed no signs of asphyxia. Birth weight was 4090 gm.; length, 54 cm.