Abstract
Pleuropulmonary blastoma (PPB) is an extremely rare and highly malignant intrathoracic tumor in children, representing a unique form of aggressive primary lung carcinoma with a strong tendency for local recurrence. In this case report, we present a two-year-old girl who has had recurrent respiratory infections since birth. A chest X-ray revealed an abnormality, prompting a referral to a surgical team, where the lesion was identified as type II PPB based on histological analysis. After completing polychemotherapy, the patient was monitored for five years, during which clinical exams and imaging showed no evidence of disease. Diagnosing PPB requires a high index of suspicion due to its aggressive yet subtle presentation, as imaging findings and clinical symptoms may be nonspecific. This report emphasizes the diagnostic and management challenges of type II PPB, underscoring the importance of a multidisciplinary approach in low-resource settings. We explore potential prognostic factors, discuss treatment options, and propose individualized therapeutic strategies.