We report an 8-year-old boy with a six month history of unilateral, progressive facial atrophy, heralded by the development of a morpheaform plaque on the left cheek. The clinical features and investigations were consistent with a diagnosis of progressive hemifacial atrophy with contralateral maxillary mucocele. This case highlights the substantial overlap of progressive facial hemiatrophy with morphea, masqueraded by contralateral maxillary mucocele.