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Imaging revealed a complex encephalocele with absence or hypoplastic aspects of venous structures, including the vein of Galen and transverse and sigmoid sinus. Clinically, despite the anticipated poor prognosis, the patient remained stable after delivery, leading to a reassessment of the initial palliative care plan. Surgical reconstruction of the encephalocele was performed at 4 weeks of age. The most recent follow-up was conducted at 4 years of age. The patient demonstrated significant developmental progress including speech, language, and motor skills without complaints of headache or nausea. The presented case highlights the medical and ethical challenges physicians face while dealing with pediatric patients with severe congenital anomalies. The case emphasizes the importance of personalized care and reconsideration of long-term expectations for these conditions.<\/jats:p>","DOI":"10.1007\/s00381-026-07212-0","type":"journal-article","created":{"date-parts":[[2026,3,22]],"date-time":"2026-03-22T03:33:41Z","timestamp":1774150421000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Giant parieto-occipital encephaloceles: always a dismal prognosis? A case report and review of the literature"],"prefix":"10.1007","volume":"42","author":[{"given":"Wouter J.","family":"Dronkers","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene M. 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