Late malignant transformation of vestibular schwannoma (VS) following irradiation has previously been reported 29 times in the literature. Here, the authors report the first late malignant transformation of VS unrelated to neurofibromatosis or radiation exposure. After undergoing a near-total excision of a histologically benign VS, the patient developed malignant regrowth of the tumor remnant 42 months after the primary excision. This case challenges the dogmatic belief of absolute causality between radiation exposure and late malignant transformation of VS, and has important implications regarding future counseling and consent for the treatment of patients with VS.