RESUMEN El Sindrome de Apert llamado tambien acrocefalosindactilia tipo I, esta caracterizado por craneosinostosis, sindactilia simetrica en las cuatro extremidades, retardo mental, alteraciones cutaneas y maxilofaciales; esta ocasionado por una mutacion en el gen receptor 2 del factor de crecimiento fibroblastico FGFR2 expresandose en forma autosomico dominante (AD). CASO CLINICO: Se presenta caso de recien nacido masculino, Capurro de 38 semanas aproximadamente, con las caracteristicas fenotipicas clasicas de este sindrome: como es la acrocefalia y la sindactilia en manos y pies PALABRAS CLAVES: Acrocefalosindactilia ACS, FGFR2, AD. ABSTRACT Apert syndrome also called acrocephalosyndactyly Type I is characterized by craniosynostosis , symmetric syndactyly in all four limbs , mental retardation , skin and maxillofacial disorders ; It is caused by a mutation in the gene receptor 2 fibrobroblastico growth factor expressing FGFR2 autosomal dominant (AD ) . CASE REPORT: as is the acrocephaly and syndactyly in the hands and feet of newborn male case, Capurro of 38 weeks approximately, with classical phenotypic characteristics of this syndrome is presented as is the acrocephaly and syndactyly in hands and feet KEYWORDS: Acrocephalosyndactyly ACS , FGFR2 , AD.