Sandifer syndrome is an unusual combination of gastroesophageal reflux with or without hiatal hernia and torticollis. It can be considered to be part of an expanded syndrome of unusual presentation of gastroesophageal reflux. After successful surgical repair of the gastroesophageal reflux and the hiatus hernia, there is a gradual but permanent disappearance of the torticollis. An infant with this extremely rare syndrome is presented along with a review of the related literature.