The authors present the case-report of a girl with congenital myotonic dystrophy whom they transferred from a childrens home to the orthopaedic clinic on account of rigid pedes equinovari excavati at the age of 3 years. In this child it was an autosomal dominant form of the disease with transmission from the mother. In the clinical picture dominated in addition to pedes equinovari also psychomotor retardation and pectus excavatus. By surgery of both feet and intense rehabilitation it proved possible to verticalize the patient and teach her unaided walking. The authors draw attention to the variability of the clinicalmanifestation in the diagnosis of myotonic dystrophy and sometimes also a problematic prognosis. They emphasize the difference of surgical therapy between pes equinovarus congenitus, pes equinovarus in arthrogryposis multiplex congenita and pes equinovarus in myotonic dystrophy. They present excellent results of orthopaedic therapy in a child after 2 years and at the same time draw attention to the advancing psychosomatic development in the girl who is 5 years old by now.

        Key words: congenital myotonic dystrophy, contractures of the major joints, pes equinovarus