The pulmonary lymphatic system plays an important role in lung perfusion homeostasis. Congenital errors of lymphatic vessel development lead to primary pulmonary lymphatic disorders (lymphangiomas, lymphangiectasis, lymphatic dysplasia syndromes). Acquired disorders of the pulmonary lymphatic system occur in a variety of clinical settings (ranging from trauma to cancer) and may lead to serious pulmonary disease. Because of their scarcity and confusing and inconsistent use of terminology, these conditions are often misdiagnosed. Their management is difficult.

        Key words: Pulmonary lymphatic obstruction - Pulmonary lymphangiomatosis - Lymphedema - Lymphangioleiomyomatosis - Lymphangiosarcoma - Chylous pleural effusion - Noncardiac pulmo- nary edema