Primary ciliary dyskinesia (PCD) is a rare disorder that primarily affects the upper and lower respiratory tracts as a result of abnormal ciliary function. It is predominantly an autosomal recessive disorder. Exome sequencing of patients with a confirmed diagnosis of PCD has elucidated PCD-causing genes, leading to studies correlating genotype and ciliary ultrastructure abnormalities to phenotypic presentations. Lateralization defects such as situs inversus or heterotaxy syndromes are characterized by an abnormality in organ structure or position that includes cardiovascular malformation. Practice guidelines exist regarding diagnosis, investigations, and clinical care. General principles of management include airway clearance, infection control, management of sinus disease and fertility issues, and adherence to routine immunizations to prevent the progression of lung disease. For established lung disease, treating exacerbations with appropriate antibiotics based on respiratory cultures is important. Otolaryngology care and monitoring are likewise essential. Future directions in management revolve around gene therapies that encompass gene replacement therapy or gene editing involving the repair of mutated gene sequences. Long-term follow-up should be in a clinical center that provides comprehensive multidisciplinary care. Aerodigestive programs are beneficial in this regard, given major upper and lower airway complications of the disease as well as comorbid conditions that include gastroesophageal reflux and pulmonary aspiration, cardiovascular malformations, and sleep-disordered breathing. Moreover, PCD should be considered in the differential diagnosis of chronic cough, chronic rhinosinusitis, non-cystic fibrosis bronchiectasis, and heterotaxy syndromes.