Neurodegeneration with brain iron accumulation is a group of inherited disorders in which iron accumulates in the basal ganglia resulting in the emergence of various clinical signs pointing to the damage of the central nervous system. The main clinical features of the disease are dystonia, dysarthria, parkinsonism and spasticity, and in some forms of the disease leads to the optic atrophy nerve and retinal degeneration. The disease usually begins in childhood and then it is usually rapidly progressive. Some types of neurodegenerative diseases with brain iron accumulation have a later onset during adolescence or adulthood, and slower disease progression. Detected ten hereditary forms of neurodegeneration with brain iron accumulation and their associated disorders gene. The most common of them is a mutation of the gene encoding pantothenate kinase 2 (PANK2). Eight of the ten genetically defined types of NBIA are inherited in an autosomal recessive manner. The diagnosis is established on the basis of clinical findings and magnetic resonance imaging (MRI) showing the deposition of iron in the basal ganglia. The form associated with pantothenate kinase characteristic “eye of the tiger“ sign on the magnetic resonance image of the brain. The final diagnosis was confirmed by molecular genetic testing detectings mutations in genes that encode proteins involved in iron metabolism. Treatment is symptomatic and mainly limited to the alleviation of symptoms and prevention of secondary complications reduce the quality of life and cause premature death.