1979). Aust. Paediatr. J.. 278-280. the Aicardi syndrome in a 47, XXY male. The Aicardi syndrome is reported for the first time in a male child. His chromosomal analysis revealed a 47, XXY karyotype. This finding supports the suggestion that this condition is due to a newly mutated X-chromosomal dominant gene. The existence of a 47, XXY chromosomal constitution in this male allows heterozygous expressions of the gene as in the female.

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EDITORIAL SYNOPSIS A review of case reports from the literature and patients personally studied by the authors delineates the natural history of the condition, indicating that if early complications are successfully managed spontaneous involution of the tumour may be expected, radical surgery minimized and later cosmetic surgery achieve a satisfactory result.