Primary ciliary dyskinesia (PCD) is an autosomal-recessive inherited disease caused by mutations in genes involved in ciliary structure and function leading to impaired mucociliary clearance and repeated or chronic, usually bacterial, infections of the upper and lower airways and decreased lung function and bronchiectasis. Cytomegalovirus (CMV) is a DNA virus that usually causes subclinical infection and in 10% of the patients causes a mononucleosis-like syndrome. CMV is a causative agent of serious illness in vulnerable immunocompromised groups such as transplant recipients, patients with immunodeficiency or malignancy and neonates. Life-threatening infection due to CMV, including CMV pneumonia, is not common in immunocompetent patients. In this report we describe a case of an otherwise immunocompetent woman, suffering from PCD, who developed severe CMV pneumonia.