Microscopic polyangiitis associated with antineutrophil cytoplasmic antibodies directed against myeloperoxidase rarely affects the central nervous system, and this is common in the presence of other organ involvement. The authors report the case of a 12-year-old girl who presented with multiple acute parieto-occipital hematomas as the only manifestation of presumed microscopic polyangiitis. Early treatment with immunosuppression resulted in complete recovery and a favorable outcome.