Primary intimal sarcoma of the pulmonary artery is a rare and aggressive malignancy that arises from the intimal layer of the pulmonary artery. It typically presents with nonspecific symptoms such as dyspnea, chest pain, and hemoptysis, making early diagnosis challenging. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful in identifying the tumor's location and extent. A definitive diagnosis is established by biopsy, either via surgical resection or percutaneous needle biopsy. However, diagnosis can be difficult due to the rarity of the disease and the need for specialized expertise in interpreting pathology specimens. Treatment of primary intimal sarcoma of the pulmonary artery involves surgical resection, followed by adjuvant chemotherapy and radiation therapy. Despite aggressive treatment, the prognosis remains poor, with a median survival of approximately two years. However, early detection and aggressive multimodal therapy can improve outcomes. We hereby report a rare case of primary intimal sarcoma of the pulmonary artery and discuss its pathophysiology, presentation, diagnostic approach, and treatment options.