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RATIONALE Chronic infection with Pseudomonas aeruginosa (PsA) negatively impacts lung disease in patients with primary ciliary dyskinesia (PCD). There is currently limited evidence regarding the efficacy of PsA antibiotic eradication therapy (AET) in children with PCD. OBJECTIVE To assess the effectiveness of antibiotic eradication therapy of early Pseudomonas aeruginosa infection in children with primary ciliary dyskinesia. METHODS This is a retrospective study of paediatric PCD patients with a confirmed diagnosis according to the American Thoracic Society (ATS) guidelines at the Hospital for Sick Children between 2010 to 2022. Children with newly acquired PsA infection underwent antibiotic eradication therapy (AET) using a stepwise protocol. The protocol includes: (step 1) 28 days of tobramycin inhalation solution (TIS); (step 2) repeat TIS if culture positive after step 1; (step 3) 14 days of intravenous antibiotics followed by 28 days of TIS if culture positive after step 2; step 3 was also used for patients who presented with pulmonary exacerbation symptoms. The main outcome was a PsA negative culture based on the microbiological results of the first culture after completion of each step of treatment. RESULTS During the study period, 31 children had a new PsA infection and underwent AET. Of the 27 children who had been asymptomatic at the time of the PsA infection, a negative PsA cultures were achieved in 20/27 (74%), 1/7 (14%) and 5/6 (83%) after steps 1, 2 and 3 of AET respectively. All four symptomatic patients who initially treated with step 3 had successful clearance of PsA. The overall cumulative success rate of the protocol for negative culture post AET was 97% (30/31). For patients in whom AET was successful, the probability of staying PsA free for at least one year was 70%. CONCLUSION Antibiotic eradication therapy for early PsA infection is highly effective in PCD with sustained efficacy in most individuals. These data suggests that AET should be considered in all PCD children with early PsA infection.