We present the case of a preterm 6-month-old African American infant who developed craniosynostosis secondary to rickets. This child developed rickets and macrocephaly by the age of 6 months. His head continued to enlarge, and a 3D CT obtained when the child was 2 years old revealed metopic and bilateral coronal craniosynostosis. This CT suggested increased intracranial pressure, and therefore, corrective cranial vault reconstruction was performed. Craniosynostosis secondary to rickets is rarely reported, but since neither rickets nor craniosynostosis is a reportable disease, the exact incidence of both diseases is unknown. Craniosynostosis should be suspected in any rachitic child with an abnormal head circumference or shape and craniofacial CT evaluation should be performed, so that a corrective surgery can be performed at an appropriate age.