Trinucleotide disorders are a heterogeneous group that lead to neurodegeneration with severe motor and psychiatric impairments. Neither the clinical presentation nor the radiological signs are specific, leading to frequent misdiagnoses.Neuroradiological signs in trinucleotide disorders.A search in pubmed.gov was conducted to identify studies reflecting the current state of knowledge.An overview of the most common trinucleotide disorders and their characteristic radiological features is provided.As in many degenerative diseases, imaging represents only one piece of the puzzle and primarily provides guidance as to the diagnostic direction.