Malignant craniopharyngioma or anaplastic craniopharyngioma was first reported in 1987 by Akachi and colleagues. It has malignant clinical and histological features, remarkably rapid progression, atypical pathology like squamous cell carcinoma, and a poor prognosis. To date, 17 cases of malignant craniopharyngioma have been reported, most of which were secondary malignant tumors. In classic benign craniopharyngioma, adjunctive treatment after gross total removal is not necessary, but in cases of malignant tumors, adjunctive treatment is important.Here we report the first case of malignant craniopharyngioma treated with adjunctive gamma knife stereotactic radiosurgery and chemotherapy (carboplatin and etoposide, as well as temozolomide chemotherapy). Treatment effectively controlled progression of the tumor temporarily.Adjunctive gamma knife stereotactic radiosurgery and chemotherapy for malignant craniopharyngioma affects follow-up strategies, we propose the need for a revision to the World Health Organization classification regarding the evaluation of malignant craniopharyngioma.