Primary ciliary dyskinesia (MIM: 244400) is a rare genetic disorder affecting approximately 1 in 20,000 individuals and is caused by immotile or dyskinetic cilia. Defective ciliary function in upper and lower airways causes defective mucociliary airway clearance and subsequently chronic inflammation that regularly progresses to destructive airway disease (bronchiectasis). Established diagnostic tools include measurement of nasal nitric oxide (NO) production rate (most often found to be low, distinguishing PCD from other chronic destructive airway diseases), structural ciliary analyses for motility apparatus components using transmission electron microscopy (TEM) or high-resolution immunofluorescence microscopy (IF), and analysis of ciliary beating by high-speed videomicroscopy (HVSM).7, 8 Furthermore, dysfunctional sperm tails (flagella), a special type of motile cilia, can cause male infertility in PCD-affected individuals if the genetic defects also affect the composition or function of sperm flagella. Organ laterality defects are also observed with approximately half of PCD individuals exhibiting situs inversus totalis, where left-right visceral organ positions are completely inverted. Other laterality defects are less frequently observed in PCD and are summarized under the term situs ambiguous (SA) represented by conditions such as situs inversus of only one body cavity (thoracalis or abdominalis), right isomerism associated with asplenia, or left isomerism associated with polysplenia.9, 10 Congenital heart defects can also represent manifestations of SA including transposition of the great arteries or hypoplastic left heart.11