Methylmalonic aciduria (MMA) is an inborn error of metabolism that results in accumulation of methylmalonic acid in blood and increased excretion in urine. They are characterized by impaired conversion of methymalonyl CoA to succinyl CoA by the enzyme methylmalonyl CoA mutase resulting in accumulation of metabolites of branched chain amino acid catabolism. MMA has a wide clinical spectrum, ranging from a benign condition to fatal neonatal disease. Its onset ranges from the neonatal period to adulthood. We report a case of a day 4 old male child who presented with the complaints of respiratory distress, poor feeding, and excessive crying. Mother had a history of previous neonatal loss on day 3 of life. Diagnosis of MMA was made with the help of clinical presentation and laboratory investigations. At present universal newborn screening for metabolic disorders is not done routinely in India. Diagnosing and managing IEM in India and other developing countries is a challenge since most of the classic metabolic test are not routinely available. Many cases are asymptomatic and undetected and hence we report this case to stress the importance of including MMA in newborn screening programme for early detection and intervention.

Background: Subcutaneous fat necrosis of the newborn (SCFN) is a panniculitis characterized by the presence of violaceous subcutaneous nodules and indurated plaques on the back, buttocks, proximal extremities, or cheeks. It is a self-limiting benign condition sometimes associated with hypercalcemia that can lead to serious complications such as seizures, failure to thrive and renal failure. It usually develops in full term neonates who experienced some perinatal stress. The literature shows that hypercalcemia is found in nearly 51% of infants, with 95% of the infants developing it within 60 days of onset of skin  lesions. Clinical Description: In this case, we describe a full-term female infant presented to us with failure to thrive at 2 months of age with a postnatal history of meconium aspiration syndrome. On further evaluation, child was found to have hypercalcemia, anemia and bilateral nephrocalcinosis. Management & Outcome: The clinical findings and lab investigations were consistent with subcutaneous fat necrosis with hypercalcemia as its main complication. Child was successfully treated with oral corticosteroids. Conclusion: Being a rare clinical condition, it is important to consider it as a differential diagnosis in infants with significant perinatal stress and hence this case report.

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