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 reported in a patient with Aicardi syndrome. A variety of uncommon benign and malignant tumors have been associated with Aicardi syndrome; this literature is briefly reviewed. The increased frequency of tumors in Aicardi syndrome should be kept in mind when evaluating these patients.