von Recklinghausen syndrome is a complex genetic disorder that range from mild hardly noticed condition to an extremely terrifying progressive disorder with numerous complications and serious disfigurement of the body caused by generalized or huge growth of tumors. The condition was named after Friedrich Daniel von Recklinghausen who published in 1882 a detailed description of the disorder including information from previous literature. However, the condition has not been well described or documented in Iraqi children despite it is believed that the condition was observed. The main aim of this book is to describe a unique case of von Recklinghausen syndrome in an Iraqi child associated with right sided basal ganglion abnormalities on brain MRI. A second aim of this book is to describe the early historic documentation of the syndrome in the literature.