Granulomatosis with polyangiitis (GPA) is a multi-system necrotising vasculitis, particularly affecting small vessels. Upper respiratory tracts, lungs and kidneys are common target organs, while cardiac involvement would also be the first and rare manifestation of the disease. In GPA with cardiac involvement, structures such as the pericardium, myocardium, endocardium and conduction system could be involved. In the literature, there are reports of an association between autoimmune diseases and silica exposure. In our case, a 73-year-old sculptor with regular exposure to silica presented with pericarditis and was later diagnosed with granulomatosis with polyangiitis. This report provides additional evidence of an association between silica exposure and autoimmune vasculitis.