Grapevine trunk diseases (GTD) are currently one of the most devastating and challenging diseases in viticulture, leading to considerable yield losses and a remarkable decline in grapevine quality. The identification of the causal agents is the cornerstone of an efficient approach to fighting against fungal diseases in a sustainable, non-chemical manner. This review attempts to describe and expose the symptoms of each pathology related to GTD, the modes of transmission, and the harmfulness of recently reported agents. Special attention was given to new diagnostic tests and technologies, grapevine defense mechanisms, molecular mechanisms of endophytes fungal colonization, and management strategies used to control these threats. The present extended review is, therefore, an updated state-of-the-art report on the progress in the management of vineyards.