Malignant pleural mesothelioma (MPM) is a rare and aggressive neoplasm arising from the mesothelial cells that line the lungs and inner thoracic wall. Histologically, MPM is classified into three main subtypes: epithelioid, sarcomatoid, and biphasic. The most well-established risk factor is prolonged asbestos exposure, typically manifesting after a latency period of several decades. The most common presenting symptom is dyspnea, usually secondary to a symptomatic pleural effusion. Additional symptoms may include chest pain, cough, fatigue, early satiety, and unintentional weight loss. Although definitive diagnosis requires histopathologic confirmation via tissue biopsy, imaging, particularly computed tomography, can raise clinical suspicion and assist in evaluating disease extent. Treatment is multimodal, often involving a combination of surgery, systemic therapy, and radiation. Despite advances in management, the overall prognosis remains poor. This chapter provides an overview of the pathogenesis, diagnosis, and treatment of malignant pleural mesothelioma.