Introduction Familial pulmonary fibrosis (FPF) is defined as lung fibrosis affecting at least 2 members of the 1st degree of a family. Monogenic pulmonary fibrosis is usually investigated in this case and/or when an idiopathic interstitial lung disease occurs in a patient younger than 55 years-old, eventually associated with extra-thoracic involvement. We reviewed all FPF cases seen in our university (2 tertiary hospitals) in 2017 and 2018. Methods All FPF patients and an equal number of sporadic idiopathic pulmonary fibrosis (IPF) diagnosed between January 1st 2017 and December 31st 2018 were included in a database. Baseline characteristics, lung function tests, HRCT and histological patterns, haematological and liver features and one-year survival were studied. We used Mann-Whitney U and Chi-Square tests for statistical analysis. Our local ethics committee approved the study. Results In 2017 and 2018, 24 patients had suspected FPF and/or monogenic lung fibrosis (3.9% of all patients discussed in multidisciplinary meeting). They were significantly younger (median age at diagnosis 63 ± 11 years) as compared to the 24 sporadic IPF patients (73 ± 9 years), P = 0.002. Fifteen FPF patients (62.5%) agreed to undergo genetic testing. We identified a mutation of the telomerase complex in 6 of them (40%). Extra-pulmonary involvement was significantly associated with FPF (P = 0.02). One-year mortality was significantly higher in FPF (29%) as compared to IPF (4%), P = 0.02. Conclusion Our study confirms that FPF represents a substantial part of ILD, has a poor prognosis and is associated with extra-pulmonary involvement and younger age. Those patients require early support, multidisciplinary approach and genetic counselling.