Swyer-James syndrome is a rare disease with patients presenting with unilateral hyperlucent lungs due to hypoplasia of the pulmonary artery and bronchiolitis obliterans. A unilateral hyperlucent lung generally develops after a lower respiratory tract infection during early childhood. In extremely rare cases, an association of bronchogenic carcinoma with Swyer-James syndrome has been reported. Here we report a case of bronchogenic squamous cell carcinoma associated with Swyer-James syndrome with a relevant literature review.