Congenital anomalies of the orbital roof are rare occurrences. The case of a 2-year-old child with vertical orbital dystopia and abnormalities of the right bony orbit is presented. The patient underwent right orbital reconstruction to restore facial symmetry. A coronal approach with a frontal craniotomy was used for intracranial exposure. The abnormal angulation of the roof was corrected, and the defect was reconstructed with a split-calvarial bone graft harvested from the parietal region. The bone graft was secured with resorbable plate fixation. To preserve vision, reconstruction of this type must be done at an early age, preferably before the age of 4 years. In this patient, there is good facial symmetry and normal globe positioning 5 years after surgery.
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