Aspergilloma infection consists of a mass of fungal hyphae, inflammatory cells, fibrin, mucus, and tissue debris and can colonize lung cavities due to underlying diseases such as tuberculosis, sarcoidosis, bronchiectasis, cavitary lung cancer, neoplasms, ankylosing spondylitis, bronchial cysts, and pulmonary infarction. Here we report coinfection of pulmonary hydatid cyst and aspergilloma in a 34-year-old female who had had history of minor thalassemia and suffered from chest pain, dyspnea, non-productive cough for at least five months, and hemoptysis for 20 days. Radiographic sign showed a large cavitary lesion (5 x 6 x 6 cm) involving left lower lobe (LLL). Dichotomous septate hyphae were observed in bronchoalveolar lavage and biopsy specimens from LLL. The patient subsequently improved after combined anti-helminth therapies with albendazole (400 mg/bd) and lobectomy. According to morphological and molecular characterization, Aspergillus niger was confirmed. In vitro antifungal susceptibility tests revealed that the MIC values for the antifungals used in this case in increasing order were posaconazole (0.125 A mu g/ml), itraconazole and voriconazole (0.5 A mu g/ml), and amphotericin B (1 A mu g/ml). The minimum effective concentration for caspofungin was 0.125 A mu g/ml. Subsequently, we systematically reviewed 22 confirmed cases of pulmonary hydatid cyst and aspergilloma during a period of 19 years (1995-2014) and discussed the epidemiology, clinical features, and treatment of this disease.