To date there are only three reports of affected families in the literature [2–4].Here we describe a three generation kindred with a phenotype of interstitial lung disease (ILD) and rheumatoid factor positive (RF+) polyarticular juvenile idiopathic arthritis (JIA), segregating as a dominant trait due to a recurrently described V155M mutation in STING. This evidence concerns the gene STING1 and interstitial lung disease.