Liver diseases are the second-leading cause of death in patients with Turner syndrome (TS); however, the frequency and pathophysiology of hepatic steatosis (HS) are not yet well understood. The objective of this study is to evaluate the frequency of HS in TS patients and assess its association with anthropometric and biochemical patterns. A descriptive, cross-sectional study with an analysis of medical records of 32 patients with TS was performed. HS diagnosed by ultrasound was present in 18.8% patients, with only one of them being aged under 18 years. Comparing the group with and without HS, median age was 23 and 17.1 years, respectively (p = 0.227). A statistically significant differences with respect to dyslipidemia (p = 0.017), obesity (p = 0.037), BMI (p = 0.030), zBMI (p = 0.042), triglyceride (p = 0.005) and HbA1C (p = 0.013) was found, all being higher in the steatosis group. In contrast, the number of patients who received treatment with rhGH was higher in the group without HS (p = 0.037). Therefore, the study suggests that HS is more frequent in adults. As not all cases presented increased transaminases, we recommend the inclusion of US in the screening of HS. Furthermore, it is necessary to evaluate the metabolic and lipid profile of these patients to maintain normal values and prevent HS. The study was limited to some extent by its small sample size and retrospective nature. Therefore, there is a need for further studies to properly understand the variables that may be involved in the occurrence of HS in patients with TS.