We present a patient diagnosed with Shapiro syndrome without corpus callosum 
agenesis. A 4-year-old-girl was admitted to the hospital with complaints of 
sweating, cooling, and drowsiness that continued during the last week of her 
admission. Attacks occurred almost daily, and lasted for about 1 hour. All 
laboratory findings, as well as Holter and echocardiography results, were 
normal. Cranial magnetic resonance imaging demonstrated an intact corpus 
callosum, and electroencephalography obtained during an attack revealed normal 
findings. However, technetium 99m-labeled hexamethylpropylene amine oxime brain 
single-photon emission computed tomography indicated increased perfusion in the 
right thalamus, basal ganglia, and inferior frontal areas during a hypothermic 
period. Although oxcarbazepine reduced the frequency of attacks, they were not 
halted completely. The patient responded better to carbamazepine.