The generic term of primary lymphedema (PLE) refers to all lymphatic anomalies that involve the occurrence of swelling in any part of the body [1]. Traditionally, PLE is considered not only the form of lymphedema present at birth, but also the forms that develop later, up to puberty and sometimes even beyond. There are also cases that remain undiagnosed. The incidence or prevalence can only be estimated and it is not possible to have reliable data available.