As a worldwide challenge in the field of neurosurgery, there is no effective treatment method for pediatric skull defects repair in clinic. Currently clinical used cranioplasty materials couldn’t undergo adjustment in response to skull growth and deformation. An ideal material for pediatric cranioplasty should fulfill the requirements of achieving complete closure, good osseointegration, biodegradability and conformability, sufficient cerebral protection and optimal aesthetic, and functional restoration of calvaria. Biomimetic mineralized collagen-based bone material is a kind of material that simulates the microstructural unit of natural bone on the nanometer scale. Because of its high osteogenic activity, it is widely used in repair of all kinds of bone defects. Recently, the biomimetic mineralized collagen-based bone materials have successfully been applied for cranial regeneration and repair with satisfactory results. This review mainly introduces the characteristics of the biomimetic mineralized collagen-based bone materials, the advantages for the repair of pediatric skull defects, and the related progresses.