4 cases of cloverleaf skull syndrome are reported and analyzed after a brief review of the literature is presented. It is noted that reported cases of surgical management of this entity are extraordinarily rare. The rather characteristic displacement of intracranial contents suggests early onset of craniosynostosis (in intrauterine life) and that hydrocephalus is not an invariable complication present at the time of birth. Rather, it appears that the hydrocephalus is secondary to obliteration of the cortical subarachnoid spaces and the presence of a cranial ring. Total craniectomy proved to be a satisfactory treatment, one which was responsible for the reversal of hydrocephalus. Early detection and treatment of this rather rare malformation results in acceptable cosmetic and neurologic improvement.