Background：Juvenile myelomonocytic leukemia (JMML) is a rare hematological malignancy in young children and can only be cured through the allogeneic stem cell transplantation. Procedure：We have retrospectively analyzed the outcomes of nine children with JMML after unrelated cord blood transplantation (UCBT). Results：Eight patients who have received a myeloablative conditioning regimen of fludarabine (FLU), busulfan (BU), and cyclophosphamide (CY) have gotten engraftment. None of the nine patients has relapsed following initial UCBT. Six patients are still alive and in complete remission after UCBT with a median observation time of 43 months (range: 10–80 months). The 5-year overall (OS) and event-free (EFS) survival rates are 77.8% and 66.7%, respectively. Conclusions：This study shows that UCBT with FLU–BU–CY conditioning regimen can represent a suitable option for children with JMML.