Benign Pleomorphic Adenoma (PA) is a tumor rarely found in tracheal and laryngotracheal sites. A review of the literature published since 1922 has revealed only 30 certain cases of which 3 presented simultaneous involvement of both larynx and trachea. The present work describes the thirty-first case (the fourth with a laryngotracheal localization), diagnosed in a white, 40-year-old male who had been complaining of acute dyspnea for the last three years. Initially these symptoms had been interpreted as asthmatic crises. During one of these episodes, the patient underwent emergency tracheotomy and a laryngotracheoscopy revealed a rounded cricotracheal lesion with smooth surface and approximately 4 cm in cranio-caudal diameter. The mass occupied 90% of the air space and originated from the posterolateral right portion of the cricoid, and from the first 3 tracheal rings. CT and esophagoscopy ruled out its transmural invasion into the esophagus. Under rigid bronchoscopy, assisted NdYAG laser debulking was performed for biopsy purposes. The histological diagnosis was benign AP. For this reason a Grillo cricotracheal resection was performed with exeresis of the cricoid arch, mucosa of the cricoid plate and the first 4 tracheal rings. Reconstruction of the respiratory tract was achieved through termino-terminal cricothyrotracheal anastomosis. The initial diagnosis was confirmed and the resection edges were without evidence of neoplasm. Post-operative recovery proceeded without complications and the patient was discharged 7 days after surgery. Endoscopic and radiological follow-up after 30 months is still negative for any neoplastic recurrences. The laryngo-tracheal lumen is within the norm and cord motility has been preserved. The authors then describe the clinical, anatomopathological and radiological elements which prove useful in evaluating tracheal neoplasms and they underline the problems of differential diagnosis between benign AP and adenoid-cystic carcinoma. In addition, the various therapeutic options are discussed with special attention being focused on surgery through external approaches. Given the location of the AP described, the Grillo procedure-most commonly used in cases of inflammatory cricotracheal stenoses-proved well suited to the loco-regional control of the neoplasm. Stringent respect for some parts of this surgical technique make it possible to reduce post-operative complications to a minimum.