Abstract
Chiari type 0 malformation (CM-0), first introduced in 1998, is a new subtype of Chiari malformation with no signs of tonsillar ectopia. We present a case of CM-0 with multiple cafe au lait spots. This case presentation could indicate the possibility of an unrecognized correlation in the pathogenesis of CM-0 and formation of cafe au lait spots (isolated or as features of Legius syndrome/neurofibromatosis type 1).
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