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A 70 year-old female patient presented with fever, nausea and dyspnea. She had been receiving immunosuppressive therapy with methotrexate and prednisone for large-vessel vasculitis. The patient was shown to have coexistent Pneumocystis jiroveci pneumonia and primary cytomegalovirus (CMV) infection with presumed CMV pneumonitis and colitis. To our knowledge, this is the first case report on the occurrence of combined primary cytomegalovirus and Pneumocystis jiroveci infection in a patient with vasculitis. It illustrates the importance of being aware of the possibility of combined opportunistic infections in patients with rheumatologic diseases.