Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a heterogeneous group of rare, autoimmune conditions characterized by widespread, multisystemic inflammation of small to medium-sized blood vessels. We present a case report of a 46-year-old male with a history of prior ischemic strokes and recurrent bilateral non-granulomatous panuveitis associated with a strongly positive cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) titer. Initial treatment with steroids, methotrexate, and rituximab were ineffective, but the condition responded moderately to cyclophosphamide. This case underscores the importance of considering AAV in patients with unexplained ocular inflammation and highlights the role of c-ANCA testing in diagnosing and managing such cases, even in the absence of classic systemic symptoms.