Background/Objectives: Coexistence of systemic sclerosis and sarcoidosis is very ra-re. Both are systemic autoimmune diseases with lung involvement but with different pathogenesis. In contrast to findings of mid- to upper-lobe interstitial lung disease (ILD) or/with hilar lymphadenopathy in sarcoidosis, the most common lung manifes-tation of systemic sclerosis is lower-lobe ILD, which is typically characterized by a nonspecific interstitial pneumonia pattern. Distinction between lung involvement re-lated to each disease is crucial due to different therapeutic approach Methods We present herein a serie of three overlap cases: two with sarcoidosis onset before the diagnosis of systemic sclerosis and the other with systemic sclerosis onset before sarcoidos. Results: A review of cases of concomitant sarcoidosis and systemic sclerosis is dis-cussed, including the pathophysiology of each disease with shared pathways leading to the development of both conditions in one patient Conclusions: The systemic sclerosis-sarcoidosis overlap is a high-risk phenotype. Early recognition and a personalized, aggressive therapeutic approach are essential to alter the natural history of these two converging fibrotic and granulomatous processes.