The results showed that echinococcectomy with processing of the residual cavity with impulsive electric discharge is an alternative for pericystectomy and liver resection. Objective: Congenital peribronchial myofibroblastic tumor is a rare, solid mesenchymal tumor of the fetus and neonate, usually associated with nonimmune hydrops fetalis. We report the case with clinical, radiological and pathologic findings and review the other cases in the English language literature. Method: We present a case of CPMT, whom a right lung mass was detected in intrauterine life. 12 days after delivery by cesarean section, right lobectomy was performed. Results: The tumor was limited to lung, and composed of spindle cells, proliferating around bronchial unit. Central necrosis and 4-5/10 hpf was present. Patient is well 15 months after surgery. Conclusion: Congenital peribronchial myofibroblastic tumor is a rare solid pediatric tumor of lung which was named differently in the past, such as congenital fibrosarcoma, congenital leiomyosarcoma, congenital mesenchymal malformation of the lung, neonatal pulmonary hamartoma. albeit it resembles sarcoma with high cellularity, mitosis and necrosis features microscopically, no additional therapy to tumor resection is needed. So it is important to keep in mind this scary looking, but innocent tumor both in prenatal and postnatal evaluation.