We present a series of two cases of ANCA associated Vasculitis (AAV) presenting as Pulmonary Renal syndrome with associated renal vein thrombosis. Although there are enough evidences suggesting association of venous thrombosis with AAVs, the incidence of renal vein thrombosis is rare. Renal vein thrombosis should be ruled out in cases where there is delay in recovery of renal function in patients with AAV. Positive laboratory values for anti-Proteinase-3 (PR3) and anti-Myeloperoxidase (MPO) ANCA in the cases that presented as Rapidly Progressive Glomerulonephritis, helped in early initiation of treatment with complete recovery of Renal function.

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