Paraganglioma is one of the neuroendocrine neoplasms that occur at various sites on the body. These tumors are found in locations that parallel the sympathetic chain ganglion in the thoracolumbar regions and parasympathetic nervous system in craniosacral regions. A 60-year-old male visited our clinic for evaluation of a solitary mass on the right parietal area of the scalp. The mass was about 2.5×2 cm in size and palpable, protruding, firm, and oval. He noticed it three years ago and complained that it seemed to be growing in size. There was no other general or local symptom, such as pain, tenderness, or bleeding. In the past (11 years ago), he had undergone partial lung lobectomy for lung cancer and was told it was cured. An excision biopsy was performed. Histologically, the tissue showed aggregated polygonal cells with centrally located oval nuclei; the cells were arranged in distinctive cell balls, so-called zellballen. Atypical mitosis, no necrosis, no tumor cell spindling, no vascular invasion, a diffuse growth pattern, and a clear resection margin were noted. The results of special stain and immunohistochemical stains were negative for cytokeratin, HMB45, Carcinoembryonic antigen, CDX2, Vimentin, and Melan A; and positive for Ki-67 (> 2-3%), S-100 protein (focally), Chromogranin (3+), and reticulin (+). The histologic and immunohistochemical studies were consistent with the diagnosis of a paraganglioma.