Isothiocyanates constitute a group of heterocumulenes containing N=C=S functionality that is of immense importance in organic synthesis. The presence of carbonyl group in acyl isothiocyanates imparts unique reactivity to acyl isothiocyanates. The chemistry of acyl isothiocyanates, in particular, is very rich and diverse, and has been employed in synthesis of a number of biologically important heterocycles. This review article discusses the acyl isothiocyanate chemistry leading to the synthesis of biologically important heterocyclic skeletons. These include highly functionalized thiazoles, thiadiazoles, triazoles, benzimidazoles, dithiolane, spiro-fused oxazolines, triazines, and oxazines, etc. The role of acyl isothiocyanates as acylating agent and thiocyanate transfer reagent is also discussed.