Aplasia/agenesis of lacrimal and salivary glands is a rare congenital defect that has been associated with disturbances in fibroblast growth factor 10 (
FGF10). It can present with symptoms of congenital lacrimal obstruction, dry eye, and dry mouth. We report the ophthalmological and genetic
study of a 19-year-old woman and her relatives suffering from this syndrome. A new probably pathogenic variant is described in the
FGF10 gene.