To gain a broader appreciation of the clinical presentation, operative treatment, and outcome of fibrous dysplasia involving the calvarium in children, we retrospectively reviewed a series of cases of fibrous dysplasia involving the calvarium (4 males and 2 females) with patients’ age ranging from 5 to 12 years old. The clinical manifestation, radiographic findings, surgical treatment, outcome and follow-up were evaluated on the basis of medical records. Fibrous dysplasia in the series was monostotic, involving frontal bone (2 cases), temporal bone (1 case), parietal bone (2 cases) and occipital bone (1 case). The patients most commonly presented with enlarging mass and cosmetic complaints. The treatment given, depending on clinical presentation, was simple biopsy with conservative follow-up (2 cases) to cranial resection (4 cases). All the cases were histopathologically confirmed as fibrous dysplasia. It was demonstrated thatfibrous dysplasia involving the calvarium is a typically benign but slowly progressive disorder of bone. Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward. Surgery should be reserved for patients with functional impairment or cosmetic deformity. Because of the benign nature of the condition, the surgery itself should be contemplated with great caution in children.