BACKGROUND Pulmonary paraganglioma is a very rare condition with 40 cases reported in the literature. In the vast majority of cases the correct diagnosis could not be yielded preoperatively. METHODS We report a case of paraganglioma of the lung. Computed tomographic scan showed a solitary pulmonary nodule. Diagnostic thoracotomy was performed and a tumor in the left lower lobe was resected. Frozen section evaluation showed an epithelial tumor with neuroendocrine differentiation and low grade features. Accordingly, lobectomy was performed. The study of the paraffin-embedded specimen yielded furthermore a neuroendocrine differentiated tumor, but mitotic figures were rare. Immunhistochemically the final diagnosis paraganglioma was made. CONCLUSIONS In patients with pulmonary paraganglioma, the correct preoperative diagnosis is in general not available. Solitary pulmonary nodules or minor tumors of unknown histology are resected by wedge resection and sent to frozen section evaluation. Frozen section evaluation results in the diagnosis neuroendocrine tumor with more or less mitoses and mostly specified as carcinoid tumor. According to the literature biologic behaviour of carcinoid tumor and pulmonary paraganglioma is similar and thus the incorrect result of frozen section evaluation leads to a correct resection mode. If frozen section evaluation shows low grade features, surgical overtreatment may occur.