Takayasu arteritis is an uncommon disease with a variety of presentations. We report a case of Takayasu arteritis with a presentation of a pulmonary-renal syndrome in a 22-year-old woman. She presented in acute respiratory failure with hemoptysis and acute renal failure; interestingly, however, the renal biopsy was normal. Magnetic resonance angiography (MRA) showed significant narrowing in the distal abdominal aorta with bilateral renal and common iliac artery occlusions. Thoracic and abdominal angiogram confirmed MRA findings of type IV Takayasu arteritis. Percutaneous transluminal angioplasty of the left renal artery normalized kidney function. The initial presentation of Takayasu arteritis as a pulmonary-renal syndrome with severe acute renal failure and diffuse pulmonary hemorrhage is unusual; to our knowledge, this has not been described previously in the literature. We provide a clinical review of Takayasu arteritis and a discussion of systemic manifestations pertinent to the case.