Coexisting primary pulmonary leiomyosarcoma (PPL) with pulmonary Aspergillosis in immunocompetent patients is a rare occurrence. Here, we presented a 54-year-old woman presented with a dry cough for two months. Bronchoscopy revealed pulmonary aspergillosis. The patient was treated with antifungal therapy for one month without improvement. To evaluate further, a chest computed tomography (CT) scan showed a large heterogeneous enhancing mass in the lower lobe of the left lung with left atrium thrombosis. Computed tomography-guided biopsy was performed, and histopathology demonstrated the diagnosis of PPL. The metastasis workup staging showed multiple metastases in vertebrae, scapula, rib, and liver. The patient was treated with chemotherapy followed by tumor bed radiotherapy. Unfortunately, her general condition worsened, and she passed away with overall survival of fourteen months. In conclusion, clinicians should be alert to underlying malignant disease if airway Aspergillus infection is suspicious in patients without strong risk factors for invasive fungal infection.