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Background: The ketogenic diet (KD) is a high-fat, adequate-protein, low-carbohydrate diet. Ketogenic diet therapy (KDT) has become an important option in the management of drug-resistant epilepsy and some of the inborn errors of metabolism in children, with increasing evidence of its favorable effects. Objective: To evaluate patient's compliance to KD in children with drug resistant epilepsy (DRE) and the effect of KD on course and outcome of DRE. Methodology: This cross-sectional study included 42 children whose age ranged between 1 month and 18 years with drug resistant epilepsy (DRE) on ketogenic diet for at least 3 months. They were recruited from the Ketogenic Diet Clinic, Children's hospital, Ain Shams University, Cairo, Egypt. They were assessed for anthropometric measurements, betahydroxybutyrate (BHB) in blood, and acetone in urine, fasting lipid profile, serum random blood sugar; The frequency and severity of convulsions were assessed using chalfont severity scale before and after applying KD and correlated with the laboratory markers measured. Results: Beta hydroxybutarate levels in blood ranged from (0.1 - 4.6) achieving ketosis in 37patient (BHB>2mmol/L). As regard frequency of convulsions per day there was highly significant decrease after 3 month of KD with p < 0.001 compared to that before using the KD. On applying chalfont seizure severity scale on our patients there was highly significant decrease in the severity of the seizure with p < 0.001 after KD. Regarding the frequency of possible complications associated with KD, 69% of the patients had no complication, while 26.2% had constipation, 26.2% had hyperlipidemia, 2.4 % had vomiting, 2.4 % had kidney stones, and 2.4 % had urinary gravels. Conclusion: KD is an effective, safe, and tolerable therapy for children with DRE where significant improvement in both frequency and severity of seizures after KD were found. [ABSTRACT FROM AUTHOR]