Law enforcement responded to the fire around 12:40 a.m. in the 17800-block of West Winnebago Drive in unincorporated Grayslake.
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When sheriff's deputies arrived, a woman, 63, said she owned the house and that her son, 44, was still trapped inside the burning house.
Sheriff's deputies tried to get the man out of the fire, but they couldn't go in because of the smoke.
Shortly after, Gurnee Firefighters arrived they got into the home and found the man unconscious.
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He was taken to a hospital where he later died.
Lake County Sheriff's deputies, fire officials and the Illinois State Fire Marshal's Office are investigating.