Introduction: Apert syndrome (AS) is a craniosynostosis syndrome caused by mutations in the gene coding for the fibroblast growth factor receptor 2 (FGFR2). It is a rare autosomal dominant disorder characterized by a triad of clinical manifestations: craniosynostosis, facial dysmorphisms and syndactyly of the fingers and toes. Facial and oral abnormalities are common. Case Report: We reported a 5-year-old boy with genetically confirmed Apert syndrome. Clinical examination showed characteristic facial dysmorphism and oral manifestations. We performed a review of literature of orofacial findings in Apert syndrome. We focused on facial and oral manifestations. Results: Ten articles were reviewed. Facial findings reviewed were classified into four categories: Cranial features such as: frontal prominence, Flattened occiput, brachycephaly. Ophthalmologic abnormalities: Proptosis, Exorbitism, Hypertelorism. Nasal and earing abnormalities: Depressed nasal bridge, Bulbous nose, Low set ears.. and Front and profile view: Midface hypoplasia ,Concave profile, Cross bow-shaped lips. Syndactyly is a constant finding. Oral findings were classified into 3 categories: Maxilla features: high arched palatal vault, Pseudo-cleft. Malocclusion: teeth crowding, Retruded maxilla, Prognathic mandible, Anterior open bite, anterior and Posterior crossbite and other features, the most common of which are: delayed eruption and Poor oral hygiene. Conclusion: Typical dental and skeletal findings of Apert syndrome were observed upon extraoral and intraoral examination. Early dental check-up and management of AS is very important as a preventive option.