Fatty acid omega-hydroxylation, peroxisomal and mitochondrial fatty acid oxidation and related lipid-metabolizing enzymes are constitutive activities of mammalian cells. The past 5 years have witnessed an increased interest in the modulation of these pathways and functions by a new group of abnormal fatty acids (sulfur-substituted fatty acid analogs), due to the metabolic and nutritional aspects related to human health and disease, and possible treatment of certain inherited peroxisomal and mitochondrial disorders. The purpose of this review is to present an overview of current knowledge in the field and to provide an account of recent developments, particularly with respect to the chemical nature of the biologically active factors and their possible mechanism of action.