Abstract Although acute promyelocytic leukemia (APL) has been regarded as a serious medical emergency associated with disseminated intravascular coagulopathy or subsequent mortality, it is now considered a curable leukemia that is particularly sensitive to treatment with all- trans retinoic acid combined with chemotherapy. However, it is not clear whether additional chromosomal abnormalities in APL patients directly influence the prognosis or treatment response. ider(17)(q10)t(15;17)(q22;q21) has mostly been reported in adult APL patients, and only three cases of pediatric APL associated with ider(17)(q10)t(15;17) showing poor prognosis have been described in the literature. Here, we report the close follow-up (clinical and laboratory) data of a pediatric APL case associated with ider(17)(q10)t(15;17). This patient had APL relapse from the same clone 15 months after morphological remission. Furthermore, despite subsequent chemotherapy, the patient died 16 months after the initial APL diagnosis. Although based on a limited amount of data (four pediatric APL cases), such results in pediatric APL patients may provide important insight into the relationship between ider(17)(q10)t(15;17) and poor prognosis. However, further well-designed case-control studies are necessary to determine the treatment response and prognosis in pediatric or adult APL patients with ider(17)(q10)t(15;17).