A 31-year-old woman presented with symptoms and signs of constrictive pericarditis. She had a history of Degos' disease, a rare disorder characterized by skin and bowel lesions thought to be secondary to vasculitis. A chest roentgenogram showed extensive calcification of the pericardium. Although pleural and pericardial involvement has been reported in this disease, constrictive pericarditis is most unusual, and radiographically demonstrable calcification of the pericardium has not been reported previously.