Sphingolipids are a diverse group of lipids that play crucial roles in cellular functions and are involved in various biological processes. This review article provides an in-depth analysis of sphingolipid metabolism, focusing on the enzymes involved and their regulatory mechanisms. Sphingolipids, particularly ceramide, serve as hubs in intermediary metabolism and function. Deficiencies or overproduction of certain sphingolipids are linked to genetic and chronic diseases. The article highlights the importance of sphingolipid metabolism in genetic diseases and neurological and immune system ailments. It covers the de novo synthesis pathway, which involves enzymes like serine palmitoyltransferase (SPT), 3-ketosphinganine reductase (KDSR), ceramide synthase (CerS), and dihydroceramide desaturase (DEGS), and the catabolism of complex sphingolipids, including sphingomyelin hydrolysis by sphingomyelinases (SMases) and glycosphingolipid hydrolysis by glycosidases. The salvage pathway, which uses Cer's isozymes to produce Cer, is also discussed. The article emphasizes the role of regulatory subunits and their impact on sphingolipid metabolism, highlighting the significance of these enzymes in maintaining normal physiology and the pathophysiological conditions associated with their dysfunction.Sphingolipids are a diverse group of lipids that play crucial roles in cellular functions and are involved in various biological processes. This review article provides an in-depth analysis of sphingolipid metabolism, focusing on the enzymes involved and their regulatory mechanisms. Sphingolipids, particularly ceramide, serve as hubs in intermediary metabolism and function. Deficiencies or overproduction of certain sphingolipids are linked to genetic and chronic diseases. The article highlights the importance of sphingolipid metabolism in genetic diseases and neurological and immune system ailments. It covers the de novo synthesis pathway, which involves enzymes like serine palmitoyltransferase (SPT), 3-ketosphinganine reductase (KDSR), ceramide synthase (CerS), and dihydroceramide desaturase (DEGS), and the catabolism of complex sphingolipids, including sphingomyelin hydrolysis by sphingomyelinases (SMases) and glycosphingolipid hydrolysis by glycosidases. The salvage pathway, which uses Cer's isozymes to produce Cer, is also discussed. The article emphasizes the role of regulatory subunits and their impact on sphingolipid metabolism, highlighting the significance of these enzymes in maintaining normal physiology and the pathophysiological conditions associated with their dysfunction.