Refractory/relapsed acute myeloid leukemia is an advanced hematological malignancy with a poor prognosis in pediatric. The therapeutic options are limited and has been slow development of therapies for this age group. A combination of Venetoclax and Azacitidine therapy in newly diagnosed elderly patients with acute myeloid leukemia and refractory/relapsed disease has achieved impressive results. Whether similar results can be attained in pediatric patients is unknown. We present a case of adolescent girl with acute myeloid leukemia secondary to myelodysplastic syndrome, who was deemed unfit to undergo conventional chemotherapy and, was treated instead with the Venetoclax and Azacitidine.