One patient with relapsed refractory AML and a nonsense mutation in EZH2 (D725∗) of intermediate priority, treated with the proteasome inhibitor bortezomib in combination with cytarabine, achieved complete remission followed by allogeneic stem cell transplantation (HSCT) and remains in remission after four years of follow up. The gene discussed is EZH2; the disease is acute myeloid leukemia.