Anti - HMGCR (3- hydroxy – 3- Methyl glutaryl – Coenzyme A reductase) autoantibody positive Immune – mediated necrotizing myopathy was 1st recognized in an adult patient with a history of statin exposure. The clinico pathologic spectrum of the disease is constantly updated, including in statin unexposed patients, childhood- onset Cases in pediatric and adult patient with a LGMD phenotype. HMGCR myopathy should be considered as a differential diagnosis in children with idiopathic limb girdle muscular dystrophy with silent neuromuscular family history. In 2017 at least four reports described further cases of pediatric anti- HMGCR associated necrotizing myopathies [1].