Congenital myopathies are a heterogeneous group of rare neuromuscular disorders, sometimes presenting as floppy infant syndrome but generally progressing slowly. They usually begin at birth or in childhood, rarely in adulthood. Congenital myopathies are classified according to their histological, immunohistological, and ultrastructural pattern and the underlying genetic defect. A growing number of causative genes have been identified in the last years. Several congenital myopathies reveal a remarkable phenotypic overlap. The most common congenital myopathies are the nemaline myopathies, core myopathies, centronuclear myopathies, and congenital fibre-type disproportion.