INTRODUCTION Acute myeloid leukemias with MLL rearrangements are frequently associated with myelomonocytic and monoblastic/monocytic morphology, with an increased risk of leukocytosis and leukostasis-related complications. Yet, little is known regarding the clinical presentation of adult AML patients with MLL translocations based on the specific translocation partner. PATIENTS AND METHODS Two recent AML cases with t(10;11)(p12;q23) translocations are detailed, with their shared presenting symptoms highlighted, followed by a review of the current literature. RESULTS The specific t(10;11)(p12;q23) MLL translocation is a rare recurrent translocation partner, most commonly seen in pediatric and young adult AML. A high incidence of early morbidity from leukocytosis-related complications are frequently seen, including diffuse intravascular coagulation and tumor lysis syndrome with multiorgan system failure, even without a true leukocytosis. CONCLUSION With prompt therapy and intensive supportive care first remissions are frequently attained, however, patients have a high risk of relapse, extramedullary disease, and poor long-term outcomes.