Background: Statin are Food and Drug Administration (FDA)-approved for hyperlipidemia but remain controversial in children. This study provides novel evidence of the molecular effects of atorvastatin on lipoprotein lipase (LPL) in children with refractory nephrotic syndrome, a population in which such data are limited. Objectives: This study aims to evaluate the effects of atorvastatin on serum lipoprotein lipase (LPL) levels and lipid profile parameters in children with refractory nephrotic syndrome. Methods: Randomized controlled trial (RCT) double-blind study of 31 children with hyperlipidemia and refractory nephrotic syndrome, included pre- and post-test groups. A placebo was given to the first group (n = 18 patients), while atorvastatin was administered to the treatment group (n = 13 patients). Results: Total cholesterol and Low-Density Lipoprotein (LDL) levels decreased significantly after treatment in both groups (p<0.05). In the atorvastatin group, LDL reduction was more pronounced compared to baseline. High-Density Lipoprotein (HDL) and Triglyceride (TG) levels showed no significant changes in either group (p>0.05). Lipoprotein Lipase (LPL) levels increased in the atorvastatin group and decreased in the control group; however, these changes were not statistically significant. No significant differences were observed between the atorvastatin and control groups in terms of changes in lipid profile parameters and LPL levels (p>0.05). Conclusions: Atorvastatin reduced cholesterol and LDL levels within the treatment group, although no significant differences were observed compared to the control group.