Freeman-Sheldon syndrome is a rare progressive myopathic disorder affecting the face, chest, and limbs. It is characterized by three basic abnormalities: microstomia with pouting lips, camptodactyly with ulnar deviation of the fingers, and talipes equinovarus. Presence of microstomia is always associated with practical difficulties of oral hygiene maintenance and increased susceptibility to caries. Patient as well as parental counseling is important in these cases. This case report presents a 7-year-old child with Freeman-Sheldon syndrome. Emphasis is given for the dental management of such children.