Nodular fasciitis is a relatively rare pathology, first described by Konwaler in 1955, which derives from an uncontrolled fibroblastic proliferation. Many authors have described this pathology in the literature, considering previous trauma as a predisposing condition. It predominantly afflicts males between the 2nd and 4th decade of life. A rapid growth, high cellularity and infiltrative borders are the main characteristics of this fasciitis. Even though it can be ubiquitous, the head and neck regions are affected in about 13–37% of cases. The symptoms and signs depend on the localization and size of the neoplasm in relation to the surrounding noble structures, which can suffer on account of the compressive action of the mass. The gold standard treatment is surgical removal. In a very few cases, local recurrences have been described. In this article, we present a case treated by means of a minimally invasive, endoscopically assisted, surgical approach, together with providing a comprehensive literature review that collects data about all the cases afflicting the cervicofacial region described to date. To the best of our knowledge, this is the first case of nodular fasciitis located in the submandibular region approached via an endoscopically assisted, minimally invasive, surgical technique.