A 5-year-old girl with Aicardi syndrome, choroid plexus papilloma and
multiple gastric hyperplastic polyps is reported. Gastric polyposis is
unusual in the pediatric age group and has not previously been report
ed in a patient with Aicardi syndrome. A variety of uncommon benign an
d malignant tumors have been associated with Aicardi syndrome; this li
terature is briefly reviewed. The increased frequency of tumors in Aic
ardi syndrome should be kept in mind when evaluating these patients.