Multiple primary tumors at adjacent site are rare. We report a rare case of coincidentally found nasopharyngeal cancer and ventral foramen magnum meningioma. The 68-year-old male patient presented with a year history of ataxia. Radiological examination revealed lesions in the nasopharyngeal space and ventral foramen magnum. A needle aspiration biopsy for nasopharyngeal space and surgical removal for foramen magnum lesion were performed. The pathological diagnoses were nasopharyngeal cancer and meningioma, respectively. The concomitant occurrence of these two tumors is very rare and there is no known association between these two tumors. We report a case of ventral foramen magnum meningioma simultaneously present with nasopharyngeal carcinoma.