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The following review provides up to date information about the recent progress in synthetic approaches and chemical reactivities to α-(arylhydrazono)-β-ketoaldehydes, mainly using N,N-dimethyl-β-ketoenamines as an efficient starting material. These α - (arylhydrazono)-β-ketoaldehydes have assured to be of biological interest and furnish an inlet to a new class of biologically active heterocyclic compounds for biomedical implementations. The data published until the end of 2017 have been covered.