Primary pleural angiosarcoma (PPA) is an exceedingly rare and aggressive vascular malignancy arising from endothelial cells within the pleural vasculature. Its development has been associated with several risk factors, including tobacco use, tuberculosis, prior radiation, and chronic pyothorax. Common symptoms include pleuritic chest pain, dyspnea, and hemoptysis. Imaging typically reveals hemorrhagic pleural effusions and invasive pleural masses. Definitive diagnosis relies on histopathology and immunohistochemistry. Treatment is individualized, with surgery preferred for localized disease. Chemotherapeutic regimens including doxorubicin, ifosfamide, and taxane-based combinations offer limited efficacy. Immune checkpoint inhibitors (e.g., nivolumab and ipilimumab) and tyrosine kinase inhibitors (e.g., sorafenib and pazopanib) show emerging promise based on extrapolated data. Despite these interventions, the prognosis remains poor, underscoring the need for early recognition and the development of novel therapeutic strategies. Given its aggressive nature and rarity, PPA requires high clinical suspicion, multidisciplinary evaluation, and ongoing research to improve outcomes.