The present paper aims to review the diagnosis and treatment of one case of broncholithiasis with Nocardia cyriacigeorgica infection, and the relevant literatures, and to raise the awareness of this disease. The patient was calculus body habitus. Chest computed tomography (CT) showed endobronchial high-density shadow. After broncholithiasis was confirmed by painless fiberoptic bronchoscopy, bronchoscopic lithotripsy was performed to remove the stones for pathological biopsy. At the same time, sputum smears, sputum cultures were conducted to find the pathogens. Nocardia cyriacigeorgica was successfully cultured in bronchoalveolar lavage fluid. After the administration of sensitive drugs for three months the patient was clinically recovery. Broncholithiasis with Nocardia cyriacigeorgica pneumonia is extremely rare. To treat this case the clinicians should consider that the presence of endobronchial stones may lead to structural diseases, resulting in low local immunity and causing Nocardia cyriacigeorgica infection. For such disease, it is recommended to take fiberoptic bronchoscopy, lavage fluid smear, acid-fast staining, and lavage fluid culture as soon as possible to obtain etiological evidence. After the infection is controlled, lithotripsy under bronchoscope should be performed as soon as possible to correct local structural changes and reduce the incidence of reinfection.