12year old girl, second born of second degree consanguineous marriage, with normal birth history presented with gradual onset of difficulty in walking since two years, currently being unable to walk without support. No other domains of development were affected. She was on antiepilectics from 3 to 6 years of age. On examination, B/L Ptosis was present with hypertelorism, tower skull, strabismus, microcephaly and generalized muscle wasting. CNS examination revealed hypertonia of both upper and lower limbs with brisk deep tendon jerks, right sided ankle clonus and extensor plantars. MRI Brain revealed large posterior fossa cyst communicating with slit like opening with fourth ventricle and hypoplasia of left cerebellar hemisphere. MRI C-Spine demonstrated anterior displacement of tip of odontoid process and severe compression of spinal cord, suggestive of Dandy Walker Malformation with CV Junction anomaly.