The connective tissue diseases, such as rheumatoid arthritis, Sjögren's syndrome, systemic lupus erythematosus, systemic sclerosis, and vasculitis, may cause various disorders of the peripheral nervous system. In this review, the clinical effects of the connective tissues diseases on nerve and muscle are examined with particular attention to mononeuritis multiplex, distal symmetric neuropathy, fulminant motor neuropathy, compression neuropathy, sensory neuronopathy, and trigeminal sensory neuropathy.