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Objective: To investigate the clinical features and pathogenesis of progressive osseous heteroplasia (POH) in children.

Methods: The clinical features and imaging findings of a child with POH are described, and family investigations and gene comparisons were performed, followed by a literature review.

Results: A 9-year-old female with no relevant family medical history initially presented with ectopic ossification of the skin and subcutaneous tissue of the right face that developed slowly.

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