Hydralazine is an antihypertensive medication that has been associated with drug-associated autoimmune syndromes like interstitial lung disease, pauci-immune glomerulonephritis, and hypocomplementemia. Hydralazine-induced ANCA-associated vasculitis and hydralazine induced positive antinuclear antibodies are rare. Clinical manifestations range from arthralgia, petechiae, or rash to multiorgan involvement. When presented as pulmonary-renal syndrome, it can be rapidly progressive and fatal. Here, we describe a case of hydralazine-associated vasculitis with rapidly progressive glomerulonephritis and highlight the importance of early diagnosis and prompt treatment.