BACKGROUND: Dyke-Davidoff-Masson Syndrome (DDMS) is a rarely seen clinical 
entity which is characterised by cerebral hemiatrophy, contralateral hemiparesis 
and epilepsy. Radiological features are typical, such as unilateral atrophy of 
the cerebral hemisphere and associated compensatory bone changes in the skull, 
like thickening, enlargement of the paranasal sinuses and mastoid air cells.
CASE REPORT: In this article, we report the first case of DDMS associated with 
epidermoid tumour and arachnoid cyst, who underwent operation for an epidermoid 
tumour in the inter-hemispheric region. To our knowledge, this is the first 
report of DDMS associated with multiple intracranial pathologies and this 
association has not been previously described in the literature.
CONCLUSION: Any patient who receives DDMS in the light of clinical and 
radiological findings should be investigated for concomitant pathologies. 
Different sequences of MRI may be useful in the diagnosis of other intracranial 
lesions.