The paper gives a brief review of the existing literature concerning the nocturnal myoclonus syndrome (NMS). The clinical symptomatology, criteria for differential diagnosis and the relation to the restless legs syndrome (RLS) are discussed. Recently we investigated central dopamine receptor density with I-123-labeled 3'-iodo-6-methoxybenzamide (IBZM) (a highly selective CNS D2 dopamine receptor ligand) and single photon emission tomography (SPECT) in patients with NMS and found a reduced density of dopamine D2-receptors in the striatal structures, indicating a dopaminergic dysfunction in NMS and RLS. We present a report concerning a 58-year old female with NMS-associated insomnia and present IBZM SPECTs and hypnograms before and after a 3-month treatment with L-dopa and discuss the results with regard to pathophysiological theories.