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Superior vena cava (SVC) syndrome resulting from obstruction of the blood flow to the superior vena cava is rarely reported to present with life-threatening hemoptysis. The pathogenesis and the underlying mechanism are still not well described in the literature. We report a unique case of a 27-year-old man known to have end-stage kidney disease (ESKD) on hemodialysis that presented with shortness of breath and life-threatening hemoptysis that developed during the dialysis session. Computerized tomography with contrast (CTPA) confirmed the presence of a large, calcified thrombus within the SVC along with the formation of multiple collaterals which was diagnostic for SVC syndrome. Attempts for revascularization and stenting failed, and the patient had a prolonged and stormy course while admitted, including difficult alternative dialysis access that unfortunately resulted in death eventually. Here we are highlighting the importance of recognition of hemoptysis as a presentation of SVC syndrome by explaining the underlying pathogenesis and possible management options.