Background: Antisynthetase Syndrome is associated with interstitial lung disease in adult patients, but this has not been described in children.

Methods: A 13-year-old with interstitial lung disease due to Antisynthetase Syndrome and pulmonary arterial hypertension underwent emergent bilateral lung transplantation after a rapid clinical decline.

Conclusions: We present the clinical, radiographic, and histological findings of a child with interstitial lung disease due to Antisynthetase Syndrome.