Inhalation of crystalline silica is known to result in silicosis: an irreversible, disabling and potentially fatal occupational lung disease, which is associated with a variety of pulmonary and non-pulmonary complications including autoimmunity. A potential link between silicosis and systemic lupus erythematosus (SLE) is currently recognized only in cases of acute or accelerated silicosis. We report a case of SLE, a disease which usually affects young females, arising in a male former stonemason with simple silicosis. Epidemiological and clinical literature on the association of silica exposure and development of SLE are briefly reviewed. This case report and literature review highlight the link between occupational silica exposure and autoimmune disease including SLE, establishes that even simple silicosis appears linked to development of autoimmunity and emphasizes the importance of an occupational history, especially in male patients who develop SLE.