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Intra bronchial and intra tracheal tumors are very rare in pediatric age group. Most of the literature clubs primary pulmonary tumors with these; hence actual incidences are not well defined. Amongst this mucoepidermoid carcinoma (MEC), whether low or high-grade account for only 0.1–0.5% of lung carcinomas. We report such a rare case of an eight year old girl presenting with history of recurrent lower respiratory tract infections with respiratory distress. CT scan of chest was suggestive of a mediastinal mass. She underwent rigid bronchoscopic biopsy, histopathology showed it to be mucus gland adenoma. She underwent right thoracotomy with complete excision of mass along with right lower lobectomy. Histopathology reported it to be a low-grade MEC with clear margins. She did not require any chemotherapy or radiotherapy and has no recurrence at 1-year follow up.

The association of lung agenesis with esophageal atresia (EA) & tracheoesophageal fistula (TEF) is exceedingly rare with a lethal outcome. We describe two neonates with this association who underwent primary repair shortly after birth. One of these neonates also had gross hydrocephalus, bilateral microtia and right thumb deformity. The goal in the management of these patients is early protection and preservation of respiratory units which is best accomplished by primary repair.