Background: Dandy Walker malformation is an anomaly that consists; cystic dilatation of fourth ventricule, vermian hypoplasia and hydrocephalia. Patients have developmental retardation but epileptic spasms are rare condition. This case report aims to impress the importance and rarely seen combination of Dandy Walker Malformation and epileptic spasms.

Case report: A 6-months-old boy patient applied to outpatient clinic with the complaint of flexor spasms which makes clusters for about a month.  He was born on term with spontanious vaginal delivery, 3300gr of weight. Patient had a diognosis of Dandy Walker malformation since antenatal period. Parents have no consanguinity and have one healthy daughter. On the physical examination, headlag was prominent and head circumference was 43 cm (25p). An encephalocel took part in the occipital region. MRI showed typical Dandy Walker triad and electroencephalography revealed hypsarrytmia. Intramuscular ACTH has been given and cessation of spasms was seen after second dose.

Conclusion: Children who have Dandy Walker malformation should be follow up and discuss not only for developmental retardation but also for epileptic spasms.