The antiphospholipid syndrome (APS) is characterized by thrombotic events associated with the presence of antiphospholipid antibodies. Renal involvement is a frequent feature in patients with APS. APS presenting with proteinuria showed that the renal involvement in this syndrome could also be a different form of glomerulonephritis. We describe a rare case report of pauci-immune vasculitis associated with primary APS in the absence of other underlying autoimmune disorders.