A case of a 7-year-old girl with microscopic polyangiitis (MPA) with a skin eruption characterized by maculopapular, erythematous and purpuric lesions on the face, elbows, and knees is presented. Anti-neutrophil cytoplasmic autoantibodies (ANCA) with myeloperoxidase specificity (MPO-ANCA) were identified. Chest X-ray and computed tomography scan revealed diffuse infiltrates in both lung fields, suggesting alveolar hemorrhage. Microscopic hematuria was detected but a renal biopsy showed no abnormalities. Histological  examination  of a skin biopsy from a  purpuric papule showed leukocytoclastic vasculitis of the small vessels in the entire dermis. The patient was treated with prednisolone and mizoribine, resulting in an improvement in the skin lesions except for those on the knee.