TBC proteins are classified as a group because they contain a common conserved structure TBC domain. TBC domain consists of approximately 200 amino acids and presents in many eukaryotic proteins. It is reported that TBC proteins have been shown to function as a GAP for Rab GTPase. TBC proteins catalyze the hydrolysis of GTP and promote the conversion of Rab-GTP to Rab-GDP, thus participating in the specific intracellular transport. Many TBC proteins play important roles in cellular functions in mammals, and their deletions or mutations are closely related to many diseases. It is important to systematically sort out these findings and functions of the TBC family and illuminate the significance of TBC proteins in different physiological conditions. Here we reviewed the structure and function of TBC proteins, especially the function related to to Rab small GTPases.