BACKGROUND Third epidermoid tumors are a rare finding. The appearance of these tumors often makes them difficult to diagnose, and thus they require multimodality imaging. CASE SUMMARY A 48-year-old male patient reported to our hospital with complaints of vomiting and severe headache. The patient also complained of involuntary micturition for the past five days. We used a combination of computed tomography (CT) and magnetic resonance imaging (MRI) imaging modalities to confirm the presence of a malignant epidermoid cyst arising from the third ventricle. A contrastenhanced CT of the head demonstrated minimal perilesional enhancement while an MRI revealed a large, lobulated and septated T2 hyperintense mass arising from the third ventricle. The maximum size of the lesion measured 73 mm × 65 mm × 64 mm in size. CONCLUSION Malignant epidermoid arising from the third ventricle in an adult male was reported using a combination of CT, MRI, and MR spectroscopy.