Lymphangiomas are regarded as malformations arising from sequestration of lymphatic tissue that fail to communicate with the lymphatic system. Lymphangiomatosis is defined as a pathological condition where either multiple lymphangiomas are present or multiple organ systems are involved. We report a 30-year-old male who presented with cough, hemoptysis and severe dyspnea as the result of progressive chylothorax and chylopericardium. Despite three months of treatment with total parenteral fat-free nutrition, octreotide, repeated pleurocentesis, and pleuroperitoneal shunting, a life-threatening deterioration of his disease developed. Radiologic findings included multiple bilateral cystic lesions in the mediastinum, hili, pleura and pericardium, along the lymphatic vessels. Although general experience with surgical treatment of lymphangiomatosis is limited, our patient nevertheless made an almost complete functional recovery after pulmonary lymphangiectomy. Thus, preoperative risk assessment based primarily on radiologic findings may be misleading. To our knowledge this is the first report in the literature to indicate that surgery should be an option in patients with multiorgan lymphangiomatosis.