Six mucopolysaccharidosis patients who were operated at our clinic between November 2011 and February 2018 are presented here. Suboccipital craniectomy and C1 laminectomy were performed for stenosis at the level of foramen magnum. Effective decompression was achieved. There was no complication. Significant clinical relief was obtained. To follow this patient population according to the pre-defined follow-up guidelines and refer them to surgery before neurologic decline is apparent, is very important for the outcome of the patients.

Keywords: C1 laminectomy, foramen magnum stenosis, mucopolysaccharidosis, suboccipital craniectomy