Growing skull fractures occur most commonly after head injury; however, their "craniofacial equivalents" may occur after neurosurgical or craniofacial operations in pediatric patients. Experience with five separate cases is reviewed, including one case that involved the anterior cranial base and presented with vertical dystopia and proptosis. Necessary contributing factors appear to include (1) cranial bone defect, (2) dural tear, and (3) expanding intracranial process (e.g., growth of the brain). Prompt recognition and the diagnosis of the problem are essential to prevent the development of progressive neurological complications. The pathophysiology and principles of surgical management of these complex problems are explored in detail.