The neurotrophic receptor tyrosine kinases (NTRKs) gene fusions occur in many different tumors, and tropomyosin receptor kinase (TRK) inhibitors exhibit attractive antitumor activity. This case report presents that a patient with refractory AML accompanied ETV6-NTRK3 was failed in traditional chemotherapy, then received continue remission after hematopoietic stem cell transplantation (HSCT) and maintenance therapy with entrectinib. This is the first time that TRK inhibitor has been used in patient with AML after HSCT.