Waldenström macroglobulinemia (WM) is an uncommon disease whose most common presenting features are anemia, hyperviscosity-related symptoms, B symptoms, bleeding, and neurological symptoms. Pulmonary compromise is rare, and there are a few cases reported of chylothorax as a manifestation of Waldenström macroglobulinemia. We present the case of a patient who presented with a refractory chylothorax as the initial manifestation of Waldenström macroglobulinemia.