While small calcifications of the choroid plexus are frequent, a large, single intracerebral calcification originating from the choroid plexus is rare. This report presents a 27-year-old woman who was admitted because of right temporal headache which had persisted for months. There was no neurological deficit. Computed tomography demonstrated a mass of calcium density measuring approximately 3 x 3 x 4 cm in the right temporal region, extending to the temporal skull base and to the rostral edge of the petrous bone. At surgery a very hard, poorly vascularised tumour originating from the choroid plexus of the temporal horn of the right lateral ventricle was completely removed. Histological workup yielded the diagnosis of a markedly calcified choroid plexus with no indication of neoplasia or inflammation. Physiological intracranial calcifications resulting from local tissue dystrophy are usually incidental. In the case presented here, a large intracerebral choroid plexus calcification was detected in a patient presenting with episodes of severe headache. The potential pathogenetic mechanism is discussed.