Primary ciliary dyskinesia (PCD) is a rare, genetically heterogeneous disease, 
characterized by ciliary disfunction and impaired mucociliary clearance, 
resulting in a range of clinical manifestations such as chronic bronchitis, 
bronchiectasis, chronic rhino-sinusitis, chronic otitis media, situs viscerum 
inversus in almost 40-50% of cases and male infertility. The triad situs 
viscerum inversus, bronchiectasis and sinusitis is known as Kartagener syndrome. 
Up to now little is known about genetic, diagnostic and therapeutic aspects of 
primary motile ciliary diseases in children: for this reason, diagnosis is 
generally delayed and almost all treatments for PCD are not based on randomized 
studies but extrapolated from cystic fibrosis guidelines. The aim of this review 
is to propose to pediatricians a summary of current clinical and diagnostic 
evidence to obtain better knoledwge of this condition. The earlier diagnosis and 
the right treatment are both crucial to improve the prognosis of PCD.