Laurence-Moon syndrome, which is very rare, and Bardet-Biedl syndrome, whic
h is more frequent are now well-recognized as two distinct entities in pedi
atric neurology. Bardet-Biedi syndrome includes a number of common clinical
signs it shares with Laurence-Moon syndrome but also with other syndromes,
particularly Alstrome syndrome. These signs are retinitis pigmentosa, ment
al retardation, obesity, and hypogonadism. Ophthalmological and electrophys
iological examinations are essential for confirmation and correct diagnosis
of Bardet-Biedl syndrome. We present three case histories. Our third case
illustrates the possibility of below normal yet discernable electroretinogr
am amplitudes which do not infirm the diagnosis of Bardet-Biedl syndrome.