Congenital infiltrating lipomatosis of the face is a rare condition with congenital facial asymmetry due to localized overgrowth and accumulation of mature lipocytes. We describe a boy, followed from the age of five months to ten years, with left facial overgrowth, ipsilateral macroglossia, ptosis of the left upper lip, typical asymmetric dental eruption and regional macrodontia in the left upper gumma. The hypertrophic asymmetry was proportionally unaltered with growth. Magnetic resonance imaging confirmed diffuse fatty infiltration with overgrowth of regional bony and soft tissue structures. Intracranial structures were normal as was the boy's cognitive development.
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