The Dandy-Walker malformation is a congenital brain anomaly involving the fourth ventricle and cerebellum cystic dilation of the fourth ventricle, agenesis or dysplasia of the cerebellar vermis, and obstructive hydrocephalus are the characteristic features. The patient with Dandy-Walker malformation usually presents in the first year of life and the patient with macrocephaly usually early in the first year. Besides hydrocephalus, occipital meningoceles, seizures, orofacial deformities and multiple other congenital anomalies are common presenting features. The Dandy-Walker malformation may be diagnosed by ultrasound, plain x-ray, angiography, CT scan and MRI. The anomaly is best diagnosed by ultrasound in utero and MR’s ex-utero. Dandy-Walker malformation treatment options are sparse, it varies from conservative follow-up in asymptomatic patients up to shunting the cyst, shunting the ventricles or shunting both. The further development of neuroendoscopy make third ventriculostomy or placement of a transaqueductal stent attached to a valved distal peritoneal system a treatment option.