Laryngeal paraganglioma originates in the neural crest cells in the laryngeal paraganglia. Two distinct types may be cited on the basis of clinical features, but biopsy is essential for diagnosis. By light microscopy, the Zellballen pattern appears pathognomonic. The treatment consists of surgical excision.In this article, due to the rareness of the tumour, one case of laryngeal paraganglioma is presented. The general knowledge and the available literature are reviewed. The difficulties in the differential diagnosis are stressed and treatment principles are discussed.