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Gazi Üniversitesi, Tıp Fakültesi, Beyin ve Sinir Cerrahisi Anabilim Dalı, Ankara, Türkiye Desmoplastic infantile astrocytoma is a rare and usually benign brain tumor with good prognosis that presents as a large cystic and solid supratentorial tumor with dural attachment in children under 2 years-old. Although non-infantile cases of DIA have been reported , it is usually seen in early childhood . DIA is a WHO grade I neroepithelial tumor with prominent desmoplastic stroma and neuroepithelial cells. The most common presentation is abnormal increase of head circumference with tense fontanelles due to increased intracranial pressure. Focal neurological deficit, hemiparesis and seizures may occur. DIA usually has a favorable prognosis although radiological and histological features of DIA are misleading. Total or near-total resection of the tumor is sufficient treatment for DIA in most cases and further therapy may not be needed. In this article, we present a rare case of desmoplastic infantile astrocytoma in a 7-month-old child and a review of the literature. Anahtar Kelimeler : Desmoplastic infantile astrocytoma, Brain tumor, Pediatrics, Seizure