We report on an 18-year-old girl with the coexistence of an epidermoid cyst in the cerebellopontine angle (CPA) with a pituitary adenoma. The clinical course and histopathological characteristics were reviewed. The patient presented with headache, vision loss and amenorea. Computerised tomographic (CT) scan and magnetic resonance imaging (MRI) examinations demonstrated a solid sellar lesion with supra-sellar extension. There was another mass lesion in the CPA with prepontine extension. The tumours were removed surgically. Histopathological examination revealed an epidermoid cyst in the prepontine area and a pituitary adenoma in the sellar region. This has been the second case to be reported in the literature with the coexistence of an epidermoid cyst and a pituitary adenoma.