BACKGROUND: Endobronchial neuroendocrine tumors are an incredibly rare tumor in the pediatric population, yet they represent the most common primary lung tumor in older children. Due to the rarity and often nonspecific presenting symptoms, diagnosis may be delayed or overlooked when initially evaluating a patient.

CASE REPORT: We report a case of an 11-year-old girl with a medical history significant for asthma and previous diagnosis of atypical pneumonia who presented with multiple episodes of hemoptysis and was ultimately diagnosed with a left mainstem bronchus endobronchial neuroendocrine tumor. Initial evaluation included imaging and bronchoscopy, which confirmed the presence of the obstructive endobronchial lesion in the left mainstream bronchus. This tumor was managed successfully with initial bronchoscopic debulking to relieve airway obstruction, followed by complete surgical resection for definitive treatment. No additional treatment was indicated, and the patient will receive twice yearly screening with cross-sectional imaging for monitoring.

CONCLUSIONS: Surgical resection is the preferred treatment of choice for this tumor type, as complete excision with negative margins is curative and offers good prognostic outcomes when technically feasible. This entity should be included in the differential diagnosis for children presenting with atypical pneumonia that is unresponsive to traditional medical treatment. Multidisciplinary collaboration is essential in providing a thorough diagnostic workup and ensuring an appropriate course of treatment.

Keywords: Carcinoid Tumor, neuroendocrine tumors, Pediatrics