The synthesis and biological activities of 1-β-D-arabinofuranosylcytosine (Ara C) and related compounds are reviewed and discussed under following headings : 1. Synthesis of 1-β-D-arabinofuranosylcytosine. 1-1 synthesis from uridine ; 1-2 synthesis from cytidine ; 1-3 synthesis via coupling of bases with suitably protected sugars ; 1-4 synthesis via D-arabinose oxazoline derivative. 2. Biological activities of 1-β-D-arabinofuranosylcytosine and related compounds. 2-1 antitumor and antiviral activities ; 2-2 biological activation of 1-β-D-arabionfuranosylcytosine in vivo and its mode of biological actions ; 2-3 inhibition of DNA polymerases by Ara CTP 2-4 other arabinofuranosyl nucleosides. 3. Some derivatives of 1-β-D-arabinofuranosylcytosine. 3-1 modifications on cytosine moieties; 3-2 sugar-modified derivatives; 3-3 depot form of Ara C and Ara CMP. 4. Scope and limitation of cancer chemotherapy using 1-β-D-arabinofuranosylcytosine and related compounds are also finally discussed.