Medicinal fungi, due to their high nutritional and medicinal value along with safety, are regarded as invaluable resources for the discovery of new pharmaceuticals. Naematelia aurantialba, commonly known as the golden ear mushroom, is a rare medicinal and edible fungus in China, renowned for its rich nutritional profile and safety for consumption. It has a long-standing history of use in traditional medicine, with ancient texts frequently citing its benefits for liver protection and its efficacy in alleviating cough and phlegm. Modern pharmacological studies have revealed that the N. aurantialba contains various bioactive compounds and nutritional constituents, exhibiting multiple pharmacological activities, including hypoglycemic, lipid-lowering, immunomodulatory, antioxidant, antitumor, and anticoagulant effects. This review synthesizes the current literature on the active components, medicinal and nutritional values, and the cultivation history of this species, aiming to provide a reference for the rational development of N. aurantialba in health food and medical fields.