Pleuropulmonary blastoma (PPB) is a rare dysembryonic lung neoplasm of childhood. Its aggressive nature is demonstrated by its large size and metastasis at the time of diagnosis. Here, we present a case of a 5-year-old male with a history of sudden dyspnea who was initially referred to the primary health-care center for emergency medical services. His initial chest radiography showed massive hydropneumothorax in the right pleural cavity, with gross mediastinal displacement. These clinical findings and rapidly decreasing peripheral saturation prompted the emergency team to insert a right-sided intercostal drainage tube. Initially, this step was able to stabilize the condition, but a subsequent computed tomography scan revealed a vast cystic-solid mass in the right hemithorax. After establishing the diagnosis of PPB by means of histopathology and immunohistochemistry, he was operated on urgently for cytoreductive purposes. Despite the initial macroscopic clearance, the mass recurrence occurred after 3 months, contributing to the ultimate fatal outcome for the patient. Due to the cystic-solid nature of type II/III PPB, it could be masquerading as hydropneumothorax in the initial chest X-ray, a misleading yet the sole initial presentation of this rare entity.

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