Systemic sclerosis is a multisystemic disorder characterized by fibrotic changes of the skin and a variety of internal organs, most notably the gastrointestinal tract, heart, kidney and lung. Silica has been suspected to cause systemic sclerosis identical to idiopathic form. The pathogenesis is not well understood but there is increasing evidence that immune system is involved. We report one male patient with systemic sclerosis and pneumoconiosis. He had occupational history of exposure to silica as a glass factory worker. The clinical and skin biopsy findings were compatible with systemic sclerosis and chest roentgenogram showed increased interstitial markings with multiple calcified hilar lymph node suggestive of pneumoconiosis.