Familial skull base chordoma is a rare tumor derived from the remnants of the embryonic notochord. The present study describes the clinical presentation of 4 cases of skull base chordomas in a family. A 15-year-old female received staged surgeries and was pathologically confirmed with a diagnosis of skull base chordoma. Among the patient's family, 2 members had previously undergone surgery and were pathologically confirmed with chordomas; 1 family member had also received radiation therapy. Furthermore, the patient's cousin, an 18-year-old male, was confirmed to have this condition by epipharyngoscopy. All confirmed cases within the family remained alive with the condition. A literature review of familial chordoma was undertaken and 8 chordoma pedigrees were found. Familial chordoma was rare, with an estimated rate of 0.4% in all chordomas. The skull base was the predominant location for familial chordoma. Compared with sporadic chordoma, familial chordomas were diagnosed at a younger age. The brachyury gene was strongly associated with familial chordomas, however, the exact pathogenesis and genetics mechanisms remains unclear.