Background/Aims: Lipodystrophy is a rare metabolic disorder characterized by a near-total or partial lack of subcutaneous adipose tissue and is associated with insulin resistance. We aimed to evaluate the efficacy of magnetic resonance spectroscopy (MRS) imaging to explore the fat content of the liver in patients with lipodystrophy and to determine the relationship between liver fat accumulation and clinical presentations of lipodystrophy. Materials and Methods: Between July 2014 and February 2016, 34 patients with lipodystrophy were assessed by MRS for the quantifi cation of hepatic steatosis. All patients had metabolic abnormalities associated with insulin resistance. Metabolic parameters and the MRS findings were analyzed to identify potential correlations between liver fat content and disease severity. Results: The MRS fat ratios (MRS-FRs) were markedly higher, indicating severe hepatic steatosis in lipodystrophy. Patients with gener alized and partial lipodystrophy had comparable levels of MRS-FRs, although patients with generalized lipodystrophy were significantly younger. Patients with genetic lipodystrophy had elevated MRS-FRs compared with those with acquired lipodystrophy (p=0.042). The MRS-FR was positively correlated with liver enzyme alanine aminotransferase (p=0.028) and serum adiponectin (p=0.043). Conclusion: Our data suggest that MRS might be an effective, non-invasive imaging method to quantify hepatic fat content in patients with lipodystrophy. Further studies are needed to validate the technique and threshold values, which would allow accurate comparison of data acquired by different machines and centers.