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“Chiari zero malformation” is a rare and a relatively new described condition which associates syringohydromyelia without caudal displacement of the cerebellar tonsils through the foramen magnum. We present a case of a 40 years old woman with Chiari zero malformation with both syringomyelia and syringobulbia and a good clinical and radiological outcome after posterior fossa decompression. The presence of associated syringomyelia and syringobulbia in this condition is less frequent and it usually occurs in younger patients. In our case we considered syringobulbia as being an extension of syringomyelia.