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Sütçü İmam Üniversitesi, Tıp Fakültesi, Beyin ve Sinir Cerrahisi Anabilim Dalı, Kahramanmaraş, Türkiye Cranial meningoceles and encephaloceles, the end results of anterior neuropore closure defect, are thought to be due to a developmental failure of para-axial mesoderm and are considered to be related to adherences between neuro-ectoderm and cutaneous ectoderm. These malformations are covered by normal skin and therefore it is widely believed that the sacs are developed in the post-neurulation period. Cranial meningoceles and encephaloceles cause serious morphological deformities of the head and care difficulties, necessitating surgical intervention as treatment. Encephalocele usually occur at occipital and anterior region while mid interparietal location is very rare. Among vertex or midline parietal encephalocels, the atretic form is more commonly seen than large size encephalocele. Large case series on anterior encephalocele and occipital encephaloceles are present in literature. However, large encephaloceles located in midline interparietal region are limited. In the literature, interparietal posterior fontanel seated giant encephalocele case report is already available. This is a rare case report of atypical location and we present a patient with giant encephalocele with atypical location. Anahtar Kelimeler : Giant encephalocele, Dissection, Treatment, Hemodynamics