Mesenchymal hamartoma of the chest wall is an extremely rare benign lesion arising from one or more ribs in neonates. Its incidence is estimated to be <1 case in a million in general population. It usually presents in the form of chest wall mass. Respiratory distress may be existed resulting from compression of the airways and lungs. Herein, we reported a 1-day-old neonate with respiratory distress and mild chest wall deformity. Imaging studies revealed an extrapleural solid cystic lesion in the middle zone of the left hemithorax. When her respiratory distress was managed, she underwent left thoracotomy, resection of chest wall tumor, and thoracoplasty with titanium mesh. Six months of follow-up revealed no evidence of recurrence. The findings of this case report yielded that mesenchymal hamartoma is a benign lesion presenting with aggressive clinical, radiological, and histopathological characteristics that can be mistaken for malignancy.
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