It is known that some cases of neural crest tumours are hereditary. We report the clinical and cytogenetic findings in a three-generation, extended family, four members of which developed single or multiple neural crest tumours (ganglioneuroma, ganglioneuroblastoma or neuroblastoma). To our knowledge, this is the first report of a family with three generations affected. No constitutional cytogenetic abnormality was found in the two members tested. We also review the literature on familial neural crest tumours, with emphasis on those affecting more than one generation. It is important that a detailed family history, with particular reference to tumours, is obtained in all cases of childhood cancer.