Their parents were not at home at the time and the door was locked, says the Selangor fire and rescue department, which received a distress call at 3:54am.
JBPM says the charred remains of the children were found in a room and they were believed to have been trapped during the incident.
They were identified as nine-year-old Nur Syaza and her six-year-old brother Al Qayyum Faizan.
The fire caused about 70% damage to the house.
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