Noonan syndrome is a disorder characterized by central and peripheral lymphatic conducting anomalies, leading to chylothorax, chylous ascites, and metabolic derangement. Novel imaging methods, including dynamic contrast magnetic resonance lymphangiography and intranodal lymphangiography, have allowed for increased visualization of lymphatic pathology. Severe pulmonary insufficiency and chylothoraces developed in a 61-year-old man with Noonan syndrome. Dynamic contrast magnetic resonance lymphangiography and intranodal lymphangiography demonstrated central thoracic duct (TD) occlusion. The patient’s condition significantly improved after a microsurgical TD-venous anastomosis assisted by TD catheterization for imaging guidance, resulting in decompression of the lymphatic system and resolution of the pulmonary symptoms.