A family with central nervous system tumors in five individuals in two generations is reported. Of five sisters, two had an isolated meningioma and one an ependymoma. Amongst the four children of those affected there was a son with two cerebral meningiomas and a daughter with a spinal meningioma. Signs typical of von Recklinghausen neurofibromatosis (NF1) or bilateral acoustic neurofibromatosis (NF2) were not present. The pattern is consistent with autosomal dominant inheritance with relatively high penetrance.