Abstract
Lung agenesis (LA) is a rare congenital anomaly that refers to an absence of lung tissue and bronchi. Burkholderia cepacia complex (BCC) causes a fatal acute necrotising pneumonia called Cepacia syndrome. The unique concomitant association of both these rare diseases has not been highlighted in the literature to the best of our knowledge. Here, we present a case of association of right-sided lung agenesis along with Cepacia syndrome. In our case, the Burkholderia cepacia complex was found to be a multi-drug-resistant organism. Debilitating deterioration in the oxygen saturation was observed and initiation of treatment after culture sensitivity report yielded in remission of the disease. However, due to underlying congenital agenesis, therapeutic intervention with steroids and bronchodilators led to stabilising the patient by developing oxygenation. Underlying pulmonary congenital defects predispose individuals to infections. The implementation of antimicrobial therapy must be aided with culture and sensitivity reports for better outcomes.