Objective: To explore the image features and pathogenesis of muscular dystrophy associated with central nervous lesion.Methods: One case of muscular dystrophy with central nervous lesion was reported and relevant literatures were reviewed.Results: The MRI features of muscular dystrophy were brain atrophy

agyria

pachygyria

white matter abnormalities and posterior fossa malformation.The muscular dystrophy with the central nervous lesion was due to mutation in LAMA2 gene

resulting in a primary defect of lamininα2 chain of merosin.A secondary deficiency of lamininα2 chain was found in some types of MD

such as muscle-eye-brain disease.Conclusion: Muscular dystrophy can be associated with central nervous lesions and the mechanism is the deficiency of lamininα2 chain of merosin.