A considerable number of patients suffer from genetically caused primary lymphoedema. Nevertheless, sound epidemiological data are sparse. This review summons the current knowledge on the genetic reasons and foundations. The review covers the following diseases and syndromes: Klippel-Tienaunay, Nonne-Milroy, Waver and Sotos, Lissencephalia (Norman-Roberts-Type), Buschke-Ollendorff, Noonan, Aagenaes, Meige, Lymphoedema-Distichiasis, Yellow-Nail, Lymphoedemo and Ptosis, Hypotrichosis-Lymphedemo-Teleangiectasia, Konzaki-Disease, Lymphoedema-Microcepholy (-Chorioretinopothy), unilateral Giant Breast, Hennekam, congenital recessive lymphoedema, Aarskog-S(on, OLEDAID, Lymphoedema-Hypopofathyrecidism, Campomelia (Cumming-Type), and Swyer.