ABSTRACT Spontaneous remission (SR) in pediatric acute myeloid leukemia (AML‐M5) is extremely rare and is typically characterized by short duration and high relapse risk. We report a case of AML‐M5 in a child at Yunnan Children's Medical Center who achieved SR following blood transfusions and anti‐infective therapy, without leukemia‐specific treatment, but relapsed one month later. A comprehensive literature review was also conducted to explore factors contributing to SR in pediatric leukemia, with the aim of informing treatment protocols and improving outcomes for AML patients.