Abstract Background: The clinical outcome following surgical resection of intracranial epidermoid lesions is not well documented. We describe a case series evaluating the clinical presentation and outcome following surgery in patients diagnosed with these lesions. Materials and Methods: A retrospective study conducted over a 4-year period. Results: A total 38 patients who underwent surgery for intracranial epidermoid lesions were identified. Of these, 20 were supertentorial and 18 were infratentorial lesions. 33 patients presented with headache. 10 patients with supratentorial lesions presented with seizures. Patients with suprasellar epidermoid lesions presented with headache and visual problems. 14 of the 18 patients with cerebellopontine angle lesions presented with symptoms of trigeminal neuralgia. Post-operatively, 3 patients with cerebellopontine angle tumors developed facial weakness which was transient in 2 patients but permanent in 1 patient. Visual impairment in patients with suprasellar epidermoid lesions improved post-operatively. Hormonal replacement therapy was required in 2 patients. Recovery of 4 patients was complicated by Cerebrospinal fluid (CSF) leak while meningitis developed in 2 patients. One patient operated for cerebellopontine angle epidermoid died post operatively. Conclusions: Epidermoid lesions can develop anywhere in the cranial cavity. Like other space occupying lesions they often present with symptoms of raised ICP and with specific neurological deficits depending on location of the tumour. Surgical outcome is excellent with low complication rates.