Authors report a case of growing skull fracture, unusual complication of linear skull fracture in infancy and childhood. A review of 132 cases reported in literature is done with an analysis of general characteristics of this lesion. The most common localization is parietal (50%); clinical presentation is represented by development of seizures (54 cases), focal neurological deficit (57 cases) or loss of consciousness (50 cases). In 50% of cases interval time between head injury and first symptom varies between 1 day and 1 year. After the first year of age the 34.4% of patients develop seizures and 59% present loss of consciousness. Among patients from 1 day to 6 months of age, 46% develop seizures, 38% focal neurological deficit and 21% loss of consciousness. Asymptomatic presentation is more common in fronto-parietal or fronto-parieto-occipital localizations. In parieto-occipital and occipital localization (30 cases), 13 patients (43.3%) have seizures, 36.7% a focal neurological deficit and 60% loss of consciousness. In parieto-temporal localization there is a higher probability of seizures (62.5%) and loss of consciousness (62.5%). The long-term follow-up and the functional recovery in patients which undergo surgery is linked to the clinical presentation and early diagnosis.