In this article, an interesting case of Apert syndrome in a 14-year-old boy with characteristic craniosynostosis, acrocephaly, midface hypoplasia, pharyngeal attenuation, ocular manifestations, and syndactyly of the hands and feet is presented. The case is discussed in the light of relevant literature. A precise clinical differentiation must be made since considerable overlap of the features of various other syndromes could give rise to difficulties in diagnosing this condition. Besides detection and timely recognition of the syndrome to allow adequate dental care, screening at periodic intervals is merited to improve the overall quality of life of these patients. Clinical relevance 1. This paper highlights the importance of the dentist as well as the specialist in the recognition and oral care of children with this syndrome. 2. Children with teeth of unusual anatomy present a challenge for conventional dentistry. 3. It is important for a pedodontist to evaluate and intervene the malrelationship of the jaws to reduce the complexity of further orthodontic treatment. Objectives statement: The reader should understand the clinical implications of recognition of this syndrome and provision of early treatment, with a purpose to reducing the duration and complexity of further treatment.