Persistent interstitial pulmonary emphysema (PIPE) is rare and usually related to mechanical ventilation-induced lung damage in premature infants with respiratory distress syndrome (RDS). When conservative treatments for PIPE, such as surfactant treatment, steroid therapy, or selective intubation, are ineffective, surgical intervention should be considered. Although PIPE localized in one lobe has the potential for curative resection, diffuse PIPE (DPIPE) affecting both lungs usually carries a poor prognosis. Tension pneumatocele by ipsilateral expanded cyst in DPIPE may need surgical treatment in case of emergency. We present a rare case of an extremely low birth weight infant who required surgical intervention for tension pneumatocele due to DPIPE and propose the indications for surgery of tension pneumatocele with DPIPE.