An 11-year-old boy presenting with sudden onset double vision, headache and neck pain was found to have left lateral rectus palsy, papilledema and neck rigidity. An initial diagnosis of benign intracranial hypertension was considered. However, magnetic resonance imaging (MRI) of the brain surprisingly discovered the pineal gland cyst. He underwent Krause´s procedure for the excision biopsy of the cyst. Histopathological examination confirmed epidermoid cyst. Postoperatively, he had transient ataxia and upgaze palsy but recovered well. He was asymptomatic during the first and third-month follow-ups. The case highlights the unusual presentation of a rare intracranial tumor, pineal epidermoid cyst. Neuroimaging and timely surgery lead to a good outcome.