This paper reports on the orthopaedic rehabilitation of a patient with 
Tay-syndrome. Tay-syndrome is a rare monogen-inherited ektodermal dysplastic 
syndrome with ichtyosis, fragility of the hair and physical and mental 
retardation. The congenital ichtyosis is ubiquitous. Only the skin on the 
flexion side of the extremity joints are not involved (orthocerathosis combined 
with paraceratotic strings). In this case, a young boy developed bilateral 
subluxation of the hips and was not able to stand or walk freely. Contemporary 
pre- and neonatal care has prolonged the survival of newborns with severe 
genodermatoses, including this syndrome. In this case, it has provided the 
necessity for orthopaedic treatment of the problems caused by osteosclerosis and 
muscular spasticity.