Rheumatic diseases are often complicated by lung disease, commonly presenting as interstitial lung disease (ILD), with potentially detrimental consequences for patient survival. Although less frequent in pediatric patients, pulmonary involvement may be observed in almost all childhood-onset rheumatic conditions. The development of biological disease-modifying anti-rheumatic drugs has significantly improved clinical outcomes. However, disease remission is not always complete or long-lasting, and treatment may need to be discontinued due to adverse effects. A novel class of drugs, namely Janus kinase inhibitors (JAKis), has been proposed to provide a significant survival benefit for patients with rheumatic diseases. Despite the ample literature on the efficacy and safety of JAKis in rheumatic disease, only a few studies have investigated the effectiveness of these drugs in patients with pulmonary involvement, and only two case reports have presented results in pediatric patients. We provide an overview of the rationale for using JAKis in ILDs associated with rheumatic disease and summarize the main studies evaluating their efficacy in both adult and pediatric patients. The present review highlights the need for controlled long-term studies to assess the efficacy and safety of JAKis in pediatric rheumatic disease complicated by lung disease.