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Heller syndrome is a rare heterogeneous clinical syndrome that is distinctive from autism and Asperger syndrome. Several authors have emphasized the rarity of the disorder including Fombone (2002), Burd and colleagues (1988), Charan (2012), Malhotra et al (2013), and Tapanadechopone (2015) who reported the first patient in Thailand. However, the disorder has been reported in several countries including Austria, United Kingdom, United States of America, Japan, India, Canada, Denmark, Spain, Thailand, and France. We have previously observed Heller syndrome in Iraqi patients, however, the syndrome has not been well documented or reported from Iraq in the medical literature. The main aim of this book is to describe two Iraqi patients with Heller syndrome, one of them have changes in brain MRI suggestive of cerebral vasculitis. The second aim of this book is to describe the historical documentation of the syndrome in the medical literature.