Case Study: Background: Pulmonary-renal syndrome are clinical syndromes defined by diffuse alveolar hemorrhage and glomerulonephritis. It is caused by a variety of entities most commonly by anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis and anti-glomerular basement membrane (GBM) antibody disease. IgA Nephropathy is the most common cause of primary glomerulonephritis worldwide, but pulmonary complications are rare. Case Report: We report a 55-year-old female known case of Chronic Kidney Disease secondary to IgA Nephropathy who presented with upper respiratory tract infection. She was initially managed conservatively with antibiotics; however, over the course, she developed acute respiratory distress syndrome (ARDS) and hemoptysis clinically compatible with diffuse alveolar hemorrhage and gross hematuria associated with progressive decline in renal function requiring renal replacement therapy. She was treated with Methylprednisolone pulse therapy 1g/day for three days. She responded well and was discharged stable, maintained on tapering dose of prednisone and on 2x a week hemodialysis. After 3 weeks from discharge, there was no recurrence of hemoptysis but on maintenance hemodialysis. Conclusion: This case has important clinical implications because pulmonary complications of advanced IgA with negative ANCA and anti-GBM is rare and potentially fatal. Despite of this disease having no established treatment guidelines, it was managed successfully with aggressive immunosuppression.