A 55-year-old white male, with silicosis diagnosed 10 years earlier, presented massive proteinuria with microscopic hematuria, moderate renal failure and distal polyneuropathy. Bilateral renal angiography showed multiple intraparenchymal saccular aneurysms. Renal biopsy disclosed a focal segmental necrotizing glomerulonephritis and arteriolitis. After combined corticosteroid and immunosuppressive treatment, renal function improved and remained stable 6 months later.