Introduction: Primary pulmonary leiomyosarcomas are a group of rare malignant tumors. They are subdivided into those originating from pulmonary parenchyma, bronchial tree, or pulmonary arteries. They tend to expand locally, and presentation depends on the site of the tumor. Diagnosis can be challenging. Case Presentation: A 40-year-old woman presented with dyspnea and severe sepsis due to a lung mass and postobstructive pneumonia. She was diagnosed as having bilateral endobronchial primary pulmonary leiomyosarcoma and treated with bilobectomy; however, she died shortly after surgery due to complications. Conclusion: Considering the rarity of her condition, this case offers a unique opportunity to investigate its presentation, diagnosis, and treatment strategies through a study of the literature.

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