Purpose of Review <p>we aim to carry out a systematic review to synthesize and understand the epidemiological, clinical, diagnostic markers and therapeutic patterns of interstitial lung disease in Still’s disease (ILD-SD) following the PRISMA protocol.</p> Recent Findings <p>ILD is present in 2–6% of patients with Still’s disease and can manifest as acute respiratory failure with or without Macrophage Activation Syndrome. Despite the severity of ILD-SD, there is limited knowledge about the pathophysiology, the accuracy of diagnostic methods and the potential therapeutic approaches.</p> Summary <p>In this review, we found that ILD was more common in women in the first and sixth decades of life. Dyspnea, cough, hypoxemia, and chest pain are common. Children, especially those with symptom onset before two years of age, are at a higher risk of developing ILD. Diagnostic examinations revealed opacity and thickening of the septum, pleura, and bronchial bundles, along with elevated inflammatory markers. There is no consensus on treatment, but biologics have shown promise.</p> Graphical Abstract <p></p>