Stiff Person Syndrome (SPS) is characterized by rigidity of the truncal muscles with superimposed episodic and often painful muscle spasms, heightened sensitivity to external stimuli, particularly tactile and auditory, and high–titer anti–GAD antibodies. Co–contracture of agonist and antagonist muscles and continuous involuntary firing of motor units at rest are the cardinal pathophysiological hallmarks of the disease. The symptoms range from mild to severe and can progress, resulting in significant disability. The multidisciplinary treatment of SPS is aimed at relief of the painful muscular spasms and extreme rigidity. Currently, the treatment for SPS revolves around improving the quality of life by reducing the symptoms as far as possible with the use of GABAergic agonists, such as diazepam or other benzodiazepines, steroids, plasmapheresis, and intravenous immunoglobulin (IVIG). Intrathecal baclofen therapy has been a very promising treatment option. In this article we will review the presentation, diagnosis and management of SPS.