Abstract: Objective To analyze the imaging,clinical and pathological features of 2 patients with primary biliary cirrhosis（PBC） accompanying fungal infection. Methods Imaging,clinical and pathological features of 2 patients with PBC accompanying fungal infection were retrospectively analyzed with the related literature reviewed. Results Of the 2 female patients,1 was diagnosed as PBC and 1 as Sjogren syndrome accompanying PBC.Fungal infection occurred in 2 patients after treatment with glucocorticosteroid and immunosuppressive agents.Peumocystis was detected in sputum of 1 patient by PCR and fungal infection was diagnosed in 1 patient by renal needle biopsy.The 2 patients died due to the rapid progress of their disease. Conclusion The prognosis of patients with PBC accompanying fungal infection is poor.Precautions should be concerned for those receiving long-term treatment with glucocorticosteroid and immunosuppressive agents.