Craniosynostosis, also known as craniostenosis, is a common cranial facies frontalis malformation. It can seriously affect the appearance, partly hinder the mental development, and cause severe neurological and psychological disorders. To study the genetic rule of this disease is important for its diagnosis and prevention. This paper reviews the researches and progresses of mechanisms of cranial suture fusion from perspectives of the theories of skull and cranial suture development, molecular signaling pathways and genetics.