Sphingolipids are a structural component of the cell membrane, derived from sphingosine, an amino alcohol. Its sphingoid base undergoes various types of enzymatic transformations that lead to the formation of biologically active compounds, which play a crucial role in the essential pathways of cellular signaling, proliferation, maturation, and death. The constantly growing number of experimental and clinical studies emphasizes the pivotal role of sphingolipids in the pathophysiology of cardiovascular diseases, including, in particular, ischemic heart disease, hypertension, heart failure, and stroke. It has also been proven that altering the sphingolipid metabolism has cardioprotective properties in cardiac pathologies, including myocardial infarction. Recent studies suggest that selected sphingolipids may serve as valuable biomarkers useful in the prognosis of cardiovascular disorders in clinical practice. This review aims to provide an overview of the current knowledge of sphingolipid metabolism and signaling in cardiovascular diseases.