Objective To analyze clinical characteristics and diagnosis and treatment of broncholithiasis with Aspergillus infection. Methods A case of broncholithiasis associated with Aspergillus infection in a hospital was analyzed retrospectively, relevant database was searched and literatures were reviewed. Results The main clinical manifestations of this case were cough and hemoptysis, chest CT showed mass opacity and stenosis of adjacent bronchus, with exudative focus around, and was misdiagnosed as tumor/tuberculosis with pulmonary infection. Bronchoscopy examination confirmed that it was broncholithiasis with Aspergillus infection, after anti-fungal and anti-infective treatment, the absorption of focus improved. Broncholithiasis and Aspergillus were as key words and searched, no Chinese literature was found,there were 5 foreign literatures, all were from 4 countries in Southeast Asia, most of the patients were females, most lesions were on the right side, 4 cases improved after treatment, and 1 case died. Conclusion Broncholithiasis with Aspergillus infection is rare in clinical disease, especially when hemoptysis or obstruction occurs, it is easily lead to misdiagnosis and mistreatment, bronchoscopy and chest CT are important techniques to find broncholithiasis, diagnosis mainly depends on the results of pathological examination, lobectomy and bronchoscopic lithotomy are important methods to treat the disease, necessity of adjuvant antifungal therapy needs further to be studied.