Aim and background: This case report aims to highlight the atypical presentation of ANCA-associated vasculitis, where the patient experienced simultaneous occurrences of diffuse alveolar hemorrhage (DAH) and ileal perforation. ANCA-associated vasculitis is an autoimmune disorder that can lead to inflammation and damage of small blood vessels, resulting in various clinical manifestations. DAH is a common and severe pulmonary complication, while ileal perforation is a rare and life-threatening gastrointestinal complication. Case description: A 35-year-old male patient was admitted to the ICU with fever, dyspnea, and hemoptysis. He was diagnosed with MPO-positive ANCA vasculitis based on laboratory findings, and diagnostic tests. The patient's condition rapidly deteriorated, requiring invasive ventilatory support. He was treated with a combination of cyclophosphamide, rituximab, and high-dose corticosteroids. During treatment, he developed abdominal pain, dyspnea, and hypotension, and was found to have a small bowel perforation on imaging. The patient underwent emergency exploratory laparotomy, and the perforation was surgically repaired. Conclusion: This case highlights the atypical and severe manifestations of ANCA-associated vasculitis, with the occurrence of DAH and ileal perforation. The management of such complex cases requires a multidisciplinary approach, with prompt and aggressive treatment of the vasculitis and management of the complications. Clinical significance: This case emphasizes the importance of clinicians being aware of the potential for rare and atypical manifestations of ANCA-associated vasculitis. Early recognition and prompt intervention are crucial to improve patient outcomes. The case also need for further research to better understand the pathophysiological mechanisms and establish optimal treatment protocols for these rare complications.