The lysine acetyltransferase 6A (KAT6A, MOZ, MYST3) is a member of the MYST family of protein acetyltransferases, which are essential for different biological processes such as craniofacial, embryonic, stem cell development, and hematopoiesis. KAT6A is an oncogene in human acute myeloid leukemia (AML), and KAT6A overexpression in AML is associated with metastases and poor prognoses. Furthermore, KAT6A mutations play an important role in cancer formation and progression and result in therapeutic resistance in both hematopoietic malignancies and solid tumors. In this review, we discuss the structural and biological functions of KAT6A and summarize the influence of KAT6A in the development of various tumors. In addition, the recent KAT6A/B inhibitors, their anticancer activities, challenges, and perspective of developing KAT6A/B inhibitors as anticancer drug candidates were introduced.