Abstract Syndromes with Neurodegeneration with Brain Iron Accumulation (NBIA) are a group of neurodegenerative disorders characterized by excess iron mainly in the globus pallidus and sometimes adjacent areas. They clinically present as hypo- and/or hyperkinetic movement disorders and a variable degree of pyramidal, cerebellar, peripheral nerve, autonomic, cognitive and psychiatric involvement and visual dysfunction. Several causative genes underlying NBIA have been identified which explain about 65% of cases. Pathophysiologically, many of the NBIA syndromes map into related biochemical pathways and gene networks including lipid metabolism. Treatment for NBIA disorders remains symptomatic.