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The clinicalpathological findings in a 32-year old woman with ataxia-telangiectasia are presented. This is the oldest patient with this disease to be studied thoroughly clinically and at autopsy. Multiple small gliovascular malformations in the brain and spinal cord and telangiectasis of the liver were found. Other advanced lesions of ataxia-telangiectasia are illustrated. The vascular malformations of the central nervous system and liver are unique. The patient died of a malignant lymphoproliferative disorder and had five other malignant and benign neoplasms.

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The case of a child affected with neuroblastoma, ganglioneuroma and neurofibromatosis is reported. Germinal mutation is probably the cause of this coincidence. In view of the dominant inheritance of neurofibromatosis and of pheo‐chromocytoma, the inference is made that neuroblastoma generally may be attributable to a dominant mutation which in the past has been lethal, but which, as a result of therapeutic progress, may be inherited by the offspring of survivors in the future, as has been noted for retinoblastoma.

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