Reported is the first case of syringomyelia and syringobulbia associated with Arnold Chiari I malformation in a Malaysian child. The initial complaint was that of unilateral anhidrosis of the face. The chief presenting features were dissociated sensory loss, asymmetrical weakness of the left upper limb associated with subluxation of left shoulder and signs of upper motor neurone lesion in the left lower limb. One unusual feature was the presence of vesiculation followed by ulceration of the pinnae of both ears. Magnetic resonance imaging (MRI) of the posterior fossa and cervical and thoracic cord facilitated the diagnosis and was the most useful diagnostic procedure in this child.