Background: Ependymomas usually arise from the ventricular surface, Me
thods: We report an 11-year-old female who presented with a supratento
rial ectopic ependymoma, Results: The patient presented with a two-mon
th-history of progressive headache, nausea and vomiting. Examination r
evealed papilledema, horizontal nystagmus, diplopia on upward gaze, an
d right pronator drift, CT scan showed an enhancing left precentral su
bcortical lesion measuring 3 cm in diameter with associated edema and
mass effect, Its medial border was located 3 cm from the ependymal sur
face of the ventricle, A firm tumour was dissected from the centrum se
miovale white matter, and removed in tote as confirmed on MRI. Patholo
gical examination revealed histological, immuno-histochemical and elec
tron microscopic features consistent with an ependymoma, Spine MRI and
bone marrow aspirate, as well as lumbar puncture of cytology failed t
o show any dissemination. Conclusion: From the literature review, this
represents an exceptional ependymoma located at the distance from the
ventricular system or cisterns. Different pathogenic alternatives are
discussed.