By the case report of a 7 years old girl problems and difficulties in diagnosing early manifested Chorea Huntington are discussed. Dementia, rigor and akinesis are the presenting signs; choreatic movements are usually not present. Seizures occur frequently. Family history gives important informations; the autosomal dominant gene mostly is transmitted by the father. Although knowledges of biochemical changes in movement disorders are increasing, there are presently only a few possibilities to ameliorate signs and symptoms of chorea by pharmacological measures.