Co-occurrence of different brain tumors is rarely observed, being more frequent in patients affected by genetic syndromes like phacomatoses. Different histological types of intracranial lesions may present at different times in the clinical history of the patient or, more rarely, they may occurr at the same moment. In these last cases, particularly for tumors located in adjacent areas of the brain, diagnostic difficulties may arise. Moreover, even when the correct diagnosis is established, treatment strategy becomes complex and a single staged approach could be ineffective in obtaining successful tumor removal. We report a case of simultaneous sellar-suprasellar craniopharyngioma and intradural clival chordoma, successfully treated by a single staged, extended, fully endoscopic endonasal approach, which required no following adjuvant therapy. We also discuss the potential etiopathogenesis of the two lesions, reviewing the literature.