Introduction: Endobronchial granular cell tumors are uncommon in the pediatric population. Case report: A 9-year-old female presented with respiratory failure due to an endobronchial tumor. After debulking and diagnosis, she underwent thoracotomy with right upper lobe resection and bronchoplasty. Pathology demonstrated an endobronchial S-100 negative granular cell tumor, which to our knowledge, is the first such report in the literature. Conclusion: Endobronchial granular cell tumors may cause obstructive respiratory failure, are amenable to surgery, and may be S-100 negative.
Keywords: S-100 protein; alveolar soft part sarcoma; endobronchial mass; granular cell tumor.