Two brothers with BBS10-COD (cases 40 and 41) were compound heterozygotes for novel variants: c.226C>T, p.(Leu76Phe) and c.181G>T, p.(Glu61*); whereas the third patient was homozygous for missense variant c.1120T>C, p.(Ile407Thr), which is rare and was seen once as heterozygous in a patient with BBS, but never observed as homozygous. This evidence concerns the gene BBS10 and Bardet-Biedl syndrome.