We report a pulmonary neoplasm with EGFR‐(KDD) and infantile fibrosarcoma‐like histologic features in a female infant with an unusual clinical and histologic evolution, characterized by persistent disease with morphologic features of Congenital Peribronchial Myofibroblastic Tumor (CPMT) after chemotherapy and targeted therapy. Here, EGFR is linked to lung neoplasm.