Rubinstein Taybi syndrome or Broad Thumb and Hallux syndrome is a genetic multisystem disorder characterized by facial dysmorphism, growth retardation and mental deficiency. A ten-year-old boy who had come to the Department of Pedodontics, Ragas Dental College, Chennai, with the chief complaint of unaesthetic appearance with extra teeth revealed delay in the developmental milestones. The patient appeared to be hyperactive and mentally retarded. Extra oral features revealed distinctive facial appearance with a broad fore head, hypertelorism, broad nasal bridge and beaked nose. Intra oral features observed were talons cusps in the upper central and lateral incisors, mesiodens, carious teeth and plaque accumulation. Since the patient was hyper active and mentally retarded, the dental treatment was done under GA. The treatment plan and dental management of this patient are discussed in this case report.