Laryngeal tumors originating in the nerves are extremely rare, particularly in infants. Indeed, this type of neoplasm normally arises in adult males. Only 10 cases have been described in the 5 to 17 year age range. The present work reports a clinical case of granulous tumor of the larynx in an 11-year-old boy. Histologically this tumor showed cells which preserved their original structure and were in close relation with the nerve fibers, but not with the myocytes. Their metabolic activity was similar to that of the nerve cells. The neurogenic origin of the neoplastic granulous cells shows the presence of neuroectodermic cytoplasmic S-100 specific for cells derived from the neural crest. The boy manifested ingravescent dyspnea due to the presence of a myoblastoma in the right laryngeal ventricle. A thyrotomic approach was adopted because of the significant size of the neoplasm although it was possible to perform conservative surgery given that the lesion was benign. The authors underline the polymorphic histology of this neoplasm and the importance of the type of surgery which should both avoid widespread demolition since the neoplasm is benign and, at the same time, prevent recurrences.