Anti-neutrophil cytoplasmic antibody (ANCA) -positive vasculitis is a small-vessel vasculitis that affects multiple body systems. Salivary gland involvement in ANCA-associated vasculitis is rare. When present, it mimics infection or malignancy, which might lead to misdiagnosis. In this report, we describe a 72-year-old man who presented with parotid and submandibular gland pain and swelling in addition to dry mouth and eyes. He had bilateral non-tender parotid gland lumps and no lymphadenopathies. Laboratory tests were positive for ANCA, hematuria, and proteinuria but negative for Anti-Ro and -La. He was treated with corticosteroids and cyclophosphamide for acute kidney injury. Unfortunately, the patient died a few months later. This case report sheds light on a rare manifestation of salivary gland involvement in ANCA-associated vasculitis that mimics Sjögren syndrome and the challenges associated with its diagnosis and treatment.