Shapiro's Syndrome

Shapiro's syndrome(SS) is an extremely rare disease consisting of paroxysmal hypothermia, hyperhidrosis and [1] corpus callosum agenesis with onset typically on adulthood. We reported a case of 15 year old male presented with classical triad of sudden onset of headache associated with episodes of profuse sweating, low body temperature and excessive shaking/shivering. On MR imaging complete agenesis of corpus callosum with colpocephaly, dilated and highly placed 3rd ventricle. Nasal encephalocele and anterior linear falcine lipoma is seen in this case which had not been recorded in previously reported case