Unlike Goodpasture's syndrome with diffuse alveolar hemorrhage (DAH), there are scarce reports on the use of plasmapheresis for patients with a recurrence of DAH associated with antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis (AAV) on hemodialysis. We report a case of a relapse of perinuclear-AAV with DAH, five months after starting hemodialysis. The patient received apheresis and induction immunosuppressive therapy, added to a short course of daily hemodialysis treatments. The DAH resolved with seven apheresis procedures and there were no adverse effects. We suggest that patients on hemodialysis with a relapse of AAV and DAH would benefit from the prompt initiation of apheresis in combination with aggressive immunosuppressive therapy. Pulmonary hemorrhage is not included in the current guidelines for therapeutic apheresis; therefore, we report this case and, if warranted, propose this condition to be included in the guidelines.