Veno-occlusive disease (VOD) is the third leading cause of mortality after bone marrow transplant. Management consists of supportive care, with restricted fluids and diuretics. Most patients will recover, but approximately 25% may develop severe life threatening VOD with subsequent respiratory compromise and multiorgan failure. Orthotopic liver transplant has been attempted for a few patients with intractable VOD, but this approach is limited by availability of a cadaveric organ. We report a child who underwent a T-depleted unrelated donor bone marrow transplant for severe aplastic anemia as a manifestation of Schwachman-Diamond syndrome who developed severe VOD. She had evidence of engraftment when liver transplant was considered, and had no evidence of major organ dysfunction. The left lateral segment of her mother's liver was transplanted at day +33 following bone marrow transplantation. The child remains well ten months post-BMT and nine months after liver transplant. A related donor liver transplant may be a justifiable approach in a patient with severe VOD post-BMT.