Background: Inborn errors of metabolism are group of disorders due to one or more defective or nonfunctional enzymes or defect in transport of proteins. Asymptomatic newborn with IEM can later present with irreversible neurological damage, hence NBS is widely accepted in developed countries. In contrast, less information is available from developing countries. In view of paucity of reports on clinical presentation and basic metabolic workup in our country, we made an attempt to study the clinical and laboratory profile. Methodology; Study was a cross sectional observational study, consisting of 50 participants who were screened for Inherited Metabolic Disorders based on clinical features and significant preliminary laboratory findings by tandem mass spectrometry using their dried blood samples. Clinical and laboratory profile of diagnosed metabolic disorders were recorded. Results; Common clinical manifestations were seizures, poor feeding and vomiting. There was significant association with consanguinity, developmental delay and recurrent abortions. Signs of encephalopathy and organomegaly were common clinical finding. Abnormal blood gases and serum lactate were usual laboratory results. Of 50 cases fatty acid oxidation and mitochondrial disorders were major contributors. Of 50 children, 25 succumbed with fatty acid oxidation being major killer. Conclusion; Inherited metabolic diseases are not uncommon and diagnosis needs a high index of suspicion. Early diagnosis and treatment may be able to save a number of these children hence there is need to develop local facilities to establish the diagnosis. Further, there is need for neonatal screening programs in developing countries like India for early diagnosis and treatment.