Localization of epidermoid cysts to the pineal region is rare. The 7-year-old boy now reported presented with an 18-month history of progressive ataxia. CT and MRI scans demonstrated a 2.5x2.5 cm cyst at the pineal region with obstructive hydrocephalus. At surgery via an occipital transtentorial approach, a characteristic "pearly tumour" was encountered, and complete resection was achieved. We present the management of this child with pineal region epidermoid cyst and review 11 cases reported in the literature since 1968. In all, 8 of the 12 patients were males. The age at the time of diagnosis ranged from 7 years to 69 years. Parinaud's syndrome and hydrocephalus are the most common presenting findings. All but 1 patient underwent direct surgical resection; 1 had stereotactic decompression. Surgical treatment brought about complete resolution of the presenting symptoms and signs in 10 of the 12 cases. One patient had persistent upgaze palsy. One patient died from progression of the pineal region mass. This patient presented with hemiparesis, which is a marker of clinical aggressiveness. The authors advocate direct surgical attack as opposed to stereotactic diagnosis and aspiration to: (1) obtain maximal resection and thereby limit the potential for recurrence and delayed complications of the cyst; (2) possibly avoid shunt placement in patients who present with hydrocephalus; and (3) decrease the likelihood of sampling error.