Systemic sclerosis is a multisystemic disease of unknown origin charicterized by degenerative fibrotic and inflammatory changes in the skin, vessels, joints, muscles, and visceral organs. Involvement of the lung in systemic sclerosis is common, but pleural effusion is rare. Although vasculitis commonly accompanies many connective tissue disorders, it has been rarely reported in systemic sclerosis. A 43-year-old woman, with a 10-year history of Raynaud's phenomenon, was admitted due to right chest pain. Her hands showed diffuse thickening and swelling of skin. Chest X-ray showed pleural effusions and esophageal manometry showed hypotonic peristalsis and low lower esophageal sphincter tone compatible with scleroderma esophagus. Antinuclear antibodies were present (titer >1: 160) with a speckled pattern She was positive for rheumatoid factor, anti scl-70 and RNP antibodies, but negative for anti-Ro, La, and Sm antibodies. Histology of the pleura revealed the presence of leukocytoclastic vasculits. After administration of prednisolone 30 mg/day, her chest symptom was improved. We report a case of systemic sclerosis with pleural effusions due to leukocytoclastic vasculitis with review of the literatures.