Hypomorphic RAG1 defect in a child presented with pulmonary hemorrhage and digital necrosis.

•In the patients with atypical presentations of vasculitis, suspicion of monogenic disorders may avoid delays in diagnosis and treatment.•For the management of these patients with immune deficiency and vasculitis, the treatment should be carefully planned.•The report of patients with different clinical presentations will increase our knowledge about the phenotype and our experience for the treatment of similar patients.