[show abstract][hide abstract] ABSTRACT: Although autism is a global disorder, relatively little is known about its presentation and occurrence in many developing countries, such as Saudi Arabia. This article describes the reasons for referral and clinical characteristics of a sample of 49 children (37 males and 12 females) diagnosed with an autistic spectrum disorder at a tertiary referral center in Saudi Arabia. The diagnosis of autism was based on DSM-IV criteria supplemented by information obtained from parent and child interviews, rating scales, and examination of school and hospital records. Females were older than males at the time of referral. Eleven patients had a history of seizure disorder and one patient had a chromosome abnormality. Twenty-five patients were taking psychotropic medications and 14 patients were the product of consanguineous marriages. Clinical and research implications of these findings are discussed.