Lymphangiomatosis is a rare disease of childhood and early adulthood involving the lung and pleura. Patients with lymphangiomatosis generally present with cough, dyspnea, and recurrent pleural effusion. Hemoptysis and chylous pleural effusion may also occur. Lung disease is bilateral, and the hilum and mediastinum may be involved. Other areas of the body may be involved, including spleen and skin, and patients may have lytic bone lesions.