A nonchordomatous solitary lesion of the clival bone is an exceptionally rare pathological entity, which represents a diagnostic and a therapeutic challenge. Many diseases with extremely different biological behavior, and requiring a dedicated treatment strategy, may result in similar clinical patterns. The approach to a correct differential diagnosis has to consider the specific epidemiological, radiological, and histopathological characteristics of each pathology to minimize the diagnostic bias and to allow the planning of the best treatment strategy. Because of the rarity of these diseases, a systematical review of nonchordomatous lesions of the clival bone still lack in the literature. The purposes of this study are to provide an overview of the epidemiological, clinical, radiological, and histopathological characteristics of nonchordomatous clival lesions, with particular emphasis on diagnostic and therapeutic strategies and related clinical outcome.