Based on the presented clinicopathological and genetic data, an inherited form of limb-girdle muscular dystrophy (delta sarcoglycanopathy) characterized by severe clinical, biochemical and histopathological signs of muscle damage was diagnosed in a single Lagotto Romagnolo dog homozygous for the SGCD missense variant. This evidence concerns the gene SGCD and qualitative or quantitative defects of delta-sarcoglycan.