Most of the craniopharyngioma is considered to derive from residual epithelial cells during the craniopharyngeal canal degeneration. Meningioma accounting for the primary intracranial neoplasm is considered to be mainly derived from cells of arachnoid granulations. Nevertheless, rare cases show coexistence of craniopharyngioma and meningioma.Case 1: A 43-year-old male patient referred to the hospital due to paroxysmal headache combined with blurred vision for 1 month. On physical examination, the visual acuity of left eye was poorer than that of the right eye. The visual acuity of the right eye near the nasal part showed defect.MRI and pathological examination were performed. The patient received intracranial tumor resection. After surgery, the patient showed hormone disorder, followed by corresponding treatment. However, the patient was lost in the 6-month follow-up.Case 2: The 64-year-old male patient presented to our department due to decline of visual acuity within 1 year combined with polydipsia (5,000 ml per day), polyuria and fatigue for 6 months. On physical examination, the bilateral visual acuity showed decline, especially the temporal part which was nearly hemiscotosis. MRI was performed. The adamantinomatous craniopharyngioma was diagnosed with the HE staining findings. The patient received intracranial resection. After surgery, the patient was in a deep coma condition, and was lost in the follow-up.In this case study, we presented 2 patients with coexistence of craniopharyngioma and meningioma. In addition, a complete literature review was carried out to illustrate the studies on coexistence of craniopharyngioma and meningioma. Meanwhile, we tried to explain the possible mechanisms for such condition.