The case of a male child with a benign neurogenic laryngeal tumour caused by von Recklinghausen's disease is presented. At the age of eight years, tracheostomy was necessary. The tumour, deforming the right half of the larynx and extending into the right half of the neck, was removed three years later. Following this procedure, decannulation was possible, and the patient has been free of laryngological complaints for six years. Attention is drawn to the fact that autosomal dominantly inherited neurofibromatosis is a generalized benign tumour with a special form. If it involves any vital structure or forms a functional obstacle, meticulous surgical removal is necessary, and this provides good prospects over a long period.