Stiff-man syndrome is a rare disorder of the central nervous system characterized by painful involuntary stiffening of axial muscles accompanied by spasms. Symptomatology is often precipitated by tactile or emotional stimuli, volitional movements and startling noises. It is occasionally associated with epilepsy and endocrine disorders, including insulin-dependent diabetes mellitus. Certain diagnostic criteria are essential for the diagnosis of Stiff-man syndrome since the initial clinical manifestations are similar to those of other neuromuscular diseases. Research findings support the theory of central nervous system autoimmunity with resultant impairment of neuronal pathways as the probable pathogenesis of Stiff-man syndrome.