目的探讨不同类型的先天性肌病的超微结构变化规律。方法对５例４种不同类型的先天性肌病肌活检标本，观察其肌纤维超微结构变化。结果各种先天性肌病都有相对特征性的异常结构或变化，如杆状体、轴空、中央核、线粒体异常。不同类型的先天性肌病的超微病变可存在一定程度的相互重叠。结论不同类型的先天性肌病之间可能存在着密切的内在联系。 Objective To investigate the ultrastructural changes of different types of congenital myopathies. Methods Four cases of congenital myopathic muscle biopsy specimens from 5 cases were observed and their ultrastructural changes were observed. Results All kinds of congenital myopathies had relatively characteristic abnormal structures or changes, such as rod body, axial space, central nucleus and mitochondrial abnormalities. Different types of congenital myopathies may have some degree of superficial overlap. Conclusion There may be a close internal relationship between different types of congenital myopathies.