The incidence of growing skull fractures ranges from less than 0.05% to 1.6%. We reviewed 22 growing skull fracture patients retrospectively. There were 15 boys and seven girls ranging in age from newborn to 6 years (mean: 12.4 months) at the time of injury. Falling was the most frequent cause of injury. In total, 17 patients presented with a scalp mass. The scalp was sunken over the bone defect in three patients. Other symptoms and signs were seizure in five patients, hemiparesis in four, recurrent meningitis in one, and pulsatile exophthalmus in one. The most common location was the parietal region. The extent of dural defect was always greater than that of bony defect, except in one case that had been previously shunted for hydrocephalus. In another patient with a growing fracture in the posterior cranial fossa, the dural edges could not be exposed, although a wide craniotomy was performed. Therefore, a cystoperitoneal shunt was inserted. Gliotic tissue was present in all the patients. Cyst or cystic lesions were observed in only nine patients, duraplasty was performed in 21, 16 were neurologically intact, and six had minor deficits. All patients under the age of 3 years with a diastatic skull fracture should be closely followed up. A sustaining diastatic fracture and brain herniation through the skull defect shown on CT or MRI imply a growing skull fracture.