Hemifacial hyperplasia (HFH) is an unusual congenital anomaly of debatable aetiology. It is characterised by facial asymmetry that is caused by a marked unilateral and localised overgrowth of facial soft tissues, bones and teeth. We report a case of true HFH with typical manifestations with a brief review of the clinical features and radiological findings associated with HFH, the differential diagnoses that should be considered and the available treatment options for HFH. It is hoped that this report will supplement the existing literature on this anomaly.