We report a case of a 2-year-8-month-old female without parental consanguinity. She was vaccinated up to date according to the national immunization program. No known recent tuberculosis or personal atopy and no family history of tumors were found. She was referred for a dry cough, becoming wet, dyspnea, and chest pain with fever. The clinical examination found a conscious patient with fever at 38.5 C tachypnea at 45 breaths per minute, normal heart rate closer to 100 beats per minute. Her oxygen saturation was 98% at room air. Her body weight was 10 kilograms, and her size was 88 cm. The pleuropulmonary examination found signs of respiratory distress, such as intercostal, subcostal, and supraclavicular retractions and decrease in vocal resonance. On auscultation, there was absence of breath sounds and tympanism in the right chest. The chest X-ray showed a right pneumothorax (Figure 1) that was drained. The laboratory data showed leukocytosis at 16,000/mm3, predominantly neutrophilic at 8,360/mm3, hemoglobin at 7.9 g/dl, and CRP at 70 mg/dl, and the blood culture was sterile. The patient was treated by amoxicillin-clavulanic acid without much clinical and radiological improvement. A thoracic CT scan searching an underlying pathology revealed overdistension of the right hemithorax with parenchymal air cystic lesions involving the external segment of the middle lobe conducting to a malformation cystic adenomatoid, a fairly abundant right hydropneumothorax responsible for a compressive effect on the adjacent pulmonary parenchyma, and inflammation of the pleura (Figure 2). The patient has been scheduled for surgery; however, she was lost because of COVID-19 pandemic and social distancing, so she missed her scheduled surgery. Two months later, she presented with pyopneumothorax that was drained. The drain brought back 100 ml of yellow ladle liquid with lumps of pus. Bacteriological examination of pus isolated Pseudomonas aeruginosa and Klebsiella pneumoniae. A right middle lobectomy was performed because we suspected a cystic adenomatoid malformation of the right middle lobe. Through an open thoracotomy, we found a gelatinous component in the right chest originated from the middle right lobe extending to the pleura and diaphragm; a middle lobectomy was performed with resection of the gelatinous component (Figure 3).The specimen weighed 194 g and measured 16 cm. Cytological finding confirmed macroscopically multiple fragments with polypoid cerebroid and necrotic component and rare cystic wall.Microscopically, the tumor proliferation had the double epithelial (Figure 4) and sarcomatous component (Figure 5). We conducted type II of pneumoblastoma. The extension assessment found secondary hepatic localization. Adjuvant chemotherapy has been indicated in addition to surgery, and the child was referred to the pediatric oncology unit. Six months after the surgery, the patient developed a local recurrence that is still being treating by chemotherapy.