A case of a 25-year-old female patient with reduction of visual acuity, strabismus convergence with corneal scars and paralimbal neovascularisation of her left eye with skin changes of linear skin naevus type is presented. The diagnosis of Jadassohn-Lewandowsky syndrome was based on characteristic skin and eye disturbances. There are also typical changes in mucous membranes and central nervous system. The treatment is difficult and a certain role may be played by cosmetic and repair surgery.