Lacrimo-auriculo-dento-digital syndrome (LADD sy) involves the association of craniofacial, digital and organ anomalies. Until now 31 cases have been published confirming monogenic autosomal dominant syndrome with variable expressivity (1, 3, 10). A case of a baby girl with bronchopulmonary dysplasia and spontaneous pneumothorax diagnosed early after delivery is presented. Cystic fibrosis was eliminated after repeated examinations. At the age of 2 years genetic examination due to growth retardation was performed. Repeated dacryocystitis, lacrimal duct stenosis, identical cup-shaped ears, small dental crowns, clinodactyly and dicolored splitting nails were found in the child and her father. The above symptoms are suggestive of familial LADD syndrome so far unpublished in our literature. The cause of LADD syndrome is a mutation of an unidentified gene that may play a role of as transcription factor of early mesoectodermic structure morphogenesis.

        Key words: lacrimal duct stenosis, bronchopulmonary dysplasia, spontaneous pneumothorax, autosomal dominant LADD syndrome