BACKGROUND: Inborn errors of metabolism (IEM) comprises of a diverse group of heterogeneous disorders manifesting in paediatric population. Cases of Inborn errors of metabolism, individually are rare but collectively are common. The timing of presentation depends on significant accumulation of toxic metabolites or on the deficiency of substrate. These disorders manifest by subtle neurologic or psychiatric features often go undiagnosed until adulthood. OBJECTIVES: The objectives of the present study was to carry out preliminary screening on urine samples from pediatric population with either metabolic or neurological manifestations for inborn errors of metabolism and to know the prevalence of aminoaciduria in tertiary care setup for early diagnosis and detection. METHODS: The present study is a cross sectional time bound study carried out at Niloufer Institute of Child Health, Osmania Medical College, Hyderabad, from August 2013 to July 2014. A total of 119 samples were analyzed from suspected cases of IEM. Samples were analyzed for all physical and chemical parameters and positive cases reported by these investigations were referred for confirmation by TMS, HPLC, and GCMS. RESULTS: Among 119 children analyzed, 29 were given presumptive diagnosis of IEM based on screening tests, urinary aminoacidogram by TLC and clinical correlation. Analysis of the data showed that maximum were in the neonatal age group. Out of these 29 positive cases, 17 were generalized aminoacidurias, 2 were branched chain aminoaciduria, another 2 were methylmalonic aciduria, and one cases each of other aminoacidurias. CONCLUSION: Early recognition of IEM by screening tests in combination with strong clinical suspicion will help the clinicians to initiate prompt early treatment to prevent lethal neurological complications and developmental delay. These simple screening tests are highly cost effective and will reduce the economic burden of the patients.