Vasculitides are diseases marked by inflammation of the blood vessel walls across various organ systems. The mesenteric vasculitis (MV) affects localized mesenteric vessels of the gastrointestinal tract. It usually occurs as part of a systemic inflammatory process but could also present in isolation. There are very few published reports of isolated mesenteric artery vasculitis. Presenting symptoms often include nausea, vomiting, diarrhea, abdominal pain, rectal bleeding, often complicating the diagnostic process. Diagnosing MV as the cause of abdominal pain can be challenging, and failure to diagnose can result in significant morbidity and mortality. A timely and accurate diagnosis of MV is essential for administering the appropriate immunosuppressive therapy and preventing unnecessary surgical interventions. This review aims to provide a comprehensive discussion of MV, including its clinical presentation, diagnostic approaches, and treatment options, with a focus on achieving early diagnosis to enhance outcomes and prevent complications. Furthermore, this review addresses the diagnostic challenges associated with MV, including the lack of specific criteria and symptom overlap with other gastrointestinal disorders such as atherosclerotic mesenteric ischemia, infections, malignancies, adverse medication effects, and other vessel occlusive processes. It also emphasizes the gaps in current literature regarding optimal diagnostic strategies and the necessity for standardized treatment protocols. By addressing these gaps and challenges, we aim to optimize patient care and improve prognosis for individuals affected by MV.