Background: Neonatal seizures are a common symptom in a greatnumber of metabolic disorders, so it is important to entertain thepossibility of an IMD in any neonate presenting with seizures early anddiagnose it rapidly, allowing the opportunity for effective intervention asthere are many effective treatments, which greatly enhance the chance ofa better outcome.Objective: To establish the prevalence of inherited metabolic diseasesamong babies diagnosed with neonatal convulsions at NICU CairoUniversity, for early detection, better diagnosis and proper management,thus increasing awareness of these diseases as a cause.Methodology: The present study included 24 neonates presenting withseizures admitted at NICU Cairo University hospital during the period ofthree months from 1st June to 31th August 2009.Full history, thoroughexamination , selective metabolic screening and basic metabolicinvestigations for evaluation were done.Results: Detection of a significant rate (16.7%) with inborn error ofmetabolism. 4 babies had metabolic derangements, Biochemical geneticdiagnosis could be formulated in only 2 neonates: Propionic Acidemia,and Urea Cycle Defect.