The definition and the clinical picture of arthrogryposis multiplex congenita (AMC) are discussed and the associated anomalies, deformities and syndromes are listed. It is stressed that isolated congenital contractures in (progressive) neuromuscular disorders may be manifestations of AMC, dependent on the moment of their onset. On the issue of the etiology it is made clear that a great number of pathological processes causing immobilisation of limbs of a fetus during or shortly after the embryonic formation of joints may result in AMC. Causes of decreased fetal movement are disorders of the developing motor system on all levels and inadequacy of the environment of the fetus. Decreased fetal movements are characteristic during pregnancy. Perinatal complications of AMC are common due to malpresentation provoked by the rigid fixated joints. The incidence, heredity, therapy and prognosis are briefly reviewed. Special attention is drawn to CNS aspects: cerebral disorders causing AMC and cerebral complications resulting from the condition. Finally it is stated that the definition and classification of AMC may need further revision in the future. Moreover, it is advocated that the cerebral aspects of AMC require further study.