In neonatal disorders affecting the central nervous system, metabolic disorders are rare, but they account for a significant number of cases. Neonatal inborn errors of metabolism (IEM) are often characterized by nonspecific systemic symptoms that mimic more common acute neonatal disorders, such as sepsis, severe heart failure, and hypoxic-ischemic encephalopathy. Sepsis and cardiomyopathy may also complicate certain IEMs in the neonatal period. For long-term neurological impairments and death to be prevented, early diagnosis is imperative. Although neuroimaging findings are rarely specific, they can play a critical role in suggesting the correct diagnosis, limiting the differential diagnosis, and thereby allowing early initiation of metabolic and genetic laboratory investigations. As a newborn may present with an IEM before newborn screening results are available, neuroimaging may be particularly helpful in distinguishing metabolic disorders from other more common causes of neonatal encephalopathy, allowing for early diagnosis and treatment of IEMs based on imaging patterns. It is possible to evaluate IEMs using magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (1H-MRS). In this article, we will examine scenarios where MRI and 1H-MRS suggest or diagnose IEMs, or, alternatively, refute them

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