Milroy's disease, familial congenital lymphoedema (Meige, 1889, Nonne, 1891, Milroy, 1892) trophoedema, is a rare strictly autosomal dominant disease with varying penetration and expresivity. It is characterized by congenital lymphoedema or oedema developing soon after birth. The lymphoedema spreads slowly, sometimes in attacks, and affects mainly the lower extremities, rarely the upper ones or other sites. Later the changes imitate elephantiasis. The described case concerns twin boys born in 1963 where in 1964 Milroy's disease was diagnosed. In the investigated patient the left lower extremity was affected. The monstrously developing changes of the leg were gradually treated by the method of „shave therapy" in 1983 - 1996 six operations. Since 1990 development of malum perforans ped.l.sin., operated (by application and shift of a flap) three times. From 1987 -1998 repeatedly erysipelas (42x) and development of drug allergy. In 1986 for the first time doubts were expressed as regards the diagnosis of Milroy's disease on account of numerous clinical symptoms suggesting Recklinghausen's disease. In the brother bizarre changes in the region of the neck and right ear were found treated by surgery in 1997. Since 1987 also Recklinghausen's disease is suspected. The patient is at present a complete invalid, he does not agree with the proposed amputation.