Acrocallosal syndrome (ACS) is a rare, genetically transmitted disorder characterized by facial deformities. These include a large forehead, large anterior fontanelle, broad nasal bridge with increased intercanthal distance, partial or complete agenesis of the corpus callosum, polysyndactyly, polydactyly, and mental retardation. Limited information concerning the dental development and treatment has been published. In addition to the classic facial deformities aforementioned, the other most commonly reported oral findings are: short philtrum/upper lip (30%); high-arched palate (30%); cleft lip/palate (20%); micro/retrognathia (13%); open mouth (15%); thin lips (11%); and 1 report of over-retained primary teeth. Seizure disorders are also a common finding due to the neuroanatomical deformities associated with this disorder. The purpose of this report was to describe the case of a 7-year-old male child with acrocallosal syndrome who presented with a cleft lip and palate, hydrocephalus, a seizure disorder, and delayed exfoliation of his primary dentition and was observed for 4 years. A review is conducted to present the pertinent medical literature concerning the oral findings associated with ACS. Dental management of this case and possible contributing factors of delayed exfoliation/permanent tooth eruption are also discussed.