This report describes the pathological features in the brain of a 22 year old woman who died with Creutzfeldt-Jakob disease 8 1/2 years after completing a 4 year course of injections of human growth hormone derived from cadaver pituitaries. Spongiform change was seen in the frontal cortex. There was gliosis and neuronal loss throughout the frontal cortex, basal ganglia, thalamus, peri-aqueductal grey matter and tectum of the mid brain, pontine nuclei and dorsal medulla but no evidence of inflammation. Extensive atrophy and gliosis of the cerebellar cortex was present with widespread loss of neurons from the internal granular layer but relative sparing of Purkinje cells. Gliosis and atrophy of the mammillary bodies and gliosis of the hypothalamus may have been related to the previous surgical excision of a craniopharyngioma at the age of 2 1/2 years. The pattern of changes presented is comparable to that in other cases of Creutzfeldt-Jakob disease including those previously reported after human growth hormone administration.