Two cases of primary paragangliomas of the lung are presented. The first occurred in a 69-year-old woman and was a grossly and histologically benign tumour. The other case occurred in a 33-year-old woman who had metastases to the peribronchial lymph nodes. This seems to be the second malignant case reported. No evidence of recurrence nor metastatic disease was found at follow-up, 8 months for the first case and 7 years for the second, respectively. The morphological and immunohistochemical findings are discussed and the literature comprising 20 cases is reviewed.