Collagen VI myopathies, caused by mutations in the genes encoding collagen type VI (CoVI), represent a clinical continuum with Ullrich congenital muscular dystrophy (UCMD) and Bethlem myopathy (BM) at each end, and intermediate phenotypes in between. These disorders share common features with other muscle contracture disorders, making differential diagnosis challenging. Over the past decade, significant advances have been made in understanding the molecular pathogenesis of CoVI myopathies. Numerous mutations have been identified in the $COL6A1$, $COL6A2$, and $COL6A3$ genes, many of which are de novo and exert dominant-negative effects. Genotype-phenotype correlations have emerged, reflecting various pathogenic mechanisms: dominant de novo exon splicing enabling the synthesis and secretion of mutant tetramers, and homozygous nonsense mutations leading to premature termination and complete loss of function. This review presents the current state of diagnosis and research in CoVI myopathies, highlighting recent advances in understanding cellular functions in animal models and patient samples, particularly mitochondrial dysfunction and defects in the autophagic clearance system of skeletal muscle, which open potential therapeutic avenues.Collagen VI myopathies, caused by mutations in the genes encoding collagen type VI (CoVI), represent a clinical continuum with Ullrich congenital muscular dystrophy (UCMD) and Bethlem myopathy (BM) at each end, and intermediate phenotypes in between. These disorders share common features with other muscle contracture disorders, making differential diagnosis challenging. Over the past decade, significant advances have been made in understanding the molecular pathogenesis of CoVI myopathies. Numerous mutations have been identified in the $COL6A1$, $COL6A2$, and $COL6A3$ genes, many of which are de novo and exert dominant-negative effects. Genotype-phenotype correlations have emerged, reflecting various pathogenic mechanisms: dominant de novo exon splicing enabling the synthesis and secretion of mutant tetramers, and homozygous nonsense mutations leading to premature termination and complete loss of function. This review presents the current state of diagnosis and research in CoVI myopathies, highlighting recent advances in understanding cellular functions in animal models and patient samples, particularly mitochondrial dysfunction and defects in the autophagic clearance system of skeletal muscle, which open potential therapeutic avenues.