Growing skull fractures are rare complications of head trauma and very rarely arise in the skull base. The clinical and radiological finding and treatment of a growing fracture of the orbital roof in a 5-year-old boy are reported, and the relevant literature is reviewed. The clinical picture was eyelid swelling. Computed tomography (CT) scan was excellent for demonstrating the bony defect in the orbital roof. Frontobasal brain injury seems to play an important role in the pathogenesis of the fracture growth. Growing skull fracture of the orbital roof should be considered in the differential diagnosis in cases of persistent ocular symptoms. Craniotomy with excision of gliotic brain and granulation tissue, dural repair and cranioplasty is the treatment of choice.