Familial interstitial lung disease, particularly familial pulmonary fibrosis (FPF), is a distinct form of interstitial lung disease with a significant genetic contribution. It includes gene variants related to telomere changes, surfactant proteins, and MUC5B promoter polymorphisms that influence disease susceptibility, course, and prognosis. High-resolution computed tomography (HRCT) reveals interstitial abnormalities in at-risk relatives, especially those with pathogenic variants, allowing for early detection. Studies recommend HRCT screening from age 50 and genetic testing in those affected. Challenges remain, such as unidentified genetic variants in many cases and the psychosocial impact of screening. Future approaches combine genetics, radiology, functional tests, and biomarkers for better risk stratification and early intervention. The review integrates advances in telomere biology, surfactant genes, and MUC5B and discusses screening strategies for clinicians and researchers.