We identified two unrelated families (1, 2) with the same c.166C>T, p.(Leu56Phe) variant in the gene FGF3. Recessive variants in FGF3 have been described in patients diagnosed with LAMM syndrome, which is characterized by congenital deafness with labyrinthine aplasia (LA), microtia (M) and microdontia (M) (MIM *610706) [42]. This evidence concerns the gene FGF3 and Microdontia.