The hypothesis that exposure to silica might be associated with a wide range of autoimmune diseases including SLE (Systemic Lupus Erythematosus) has been discussed over the last decade, but only few cases of silicosis and SLE were described in the literature. We report the case of a male patient in his fifth decade of life, with previous exposure to silica, who worked as a well digger for ten years. The patient’s clinical picture started with articular symptoms, sporadic peaks of fever, anemia, positive anti-nuclear factor, peripheral (1/10) and homogeneous (1/500) standard, and productive cough. Computed tomography of the chest showed a diffuse interstitial process, bilateral nodules, para-aortic and para-tracheal hilar calcifications, compatible with pulmonary and ganglial silicosis. He developed acute respiratory distress syndrome (ARDS) and died