Resumen: Background/Objectives: Coenzyme Q 10 is a lipid molecule that works as a mobile electron transporter in the mitochondrial respiratory chain and, in addition, plays the role of an antioxidant. Interestingly, CoQ10 synthesis in human cells derives from the mevalonate pathway, the same metabolic route that delivers endogenous cholesterol. Mutations leading to Familial Hypercholesterolemia (FH) alter the levels of CoQ10 production and remarkably, statin therapy associated muscular symptoms (SAMSs) might also be modulated by CoQ10 supplementation. CoQ10 is also provided by diet and only a few studies have calculated the dietary intake of this metabolite among populations. Methods: Here, we present our Spanish FH cohort (n = 261) and characterized relevant clinical, metabolic, and anthropometric parameters. Results: A cohort of 75.1% followed lipid-lowering treatment at inclusion, being the most prescribed drugs statin alone (32.7%) and statins combined with ezetimibe (56.6%). The average time on statin treatment was 3.7 years. Interestingly, 22% of cohort patients presented with SAMS. In addition, we performed an exhaustive literature review to define for the first time the CoQ10 content present in food typically found in Spain or other southern-European countries and classified them from very rich (over 50 mg/kg) to very poor (<1 mg/kg). With this information, we calculated the daily intake of CoQ10 from a small group (12) of selected FH patients using a validated food-frequency questionnaire (FFQ) and determined a daily intake 9.72 ± 2.64 mg/day, different to other described populations. Conclusions: we discussed the relevance of exogenous CoQ10 for FH development and potential SAMS. Interestingly, this information can be extrapolated to define the regular CoQ10 intake of the Spanish population, especially when following the MedDiet. Idioma: Inglés DOI: 10.3390/nu17223512 Año: 2025 Publicado en: Nutrients 17, 22 (2025), 3512 [22 pp.] ISSN: 2072-6643 Financiación: info:eu-repo/grantAgreement/ES/MICINN/RYC2020-029544-I Tipo y forma: Artículo (Versión definitiva) Área (Departamento): Área Nutrición Bromatología (Dpto. Produc.Animal Cienc.Ali.) Área (Departamento): Area Medicina (Dpto. Medicina, Psiqu. y Derm.) Área (Departamento): Área Bioquímica y Biolog.Mole. (Dpto. Bioq.Biolog.Mol. Celular)