Diffuse pulmonary lymphangiomatosis (DPL) is a rare disorder characterized by abnormal proliferation and infiltration of lymphatic vasculature within the lungs, pleura, and mediastinum. Pleural involvement most frequently manifests as recurrent exudative effusions, with chylothorax and hemothorax also commonly reported. Radiographic findings are often nonspecific and may include mediastinal lymphadenopathy, interlobular septal thickening, ground-glass opacities, bronchial wall thickening, and pleural effusion. Histopathologic examination typically demonstrates irregular dilation and proliferation of lymphatic channels within affected tissues. Management is generally reserved for symptomatic patients and stems mainly from isolated case reports, reflecting the paucity of evidence-based data. Surgical intervention is limited to select cases due to the difficulty in distinguishing lymphatic structures from adjacent tissues; therefore, medical therapies aimed at inhibiting lymphangiogenesis are more commonly employed.