Rhabdomyomas are rare mesenchymal benign tumors of striated muscle origin that can be classified into cardiac and extracardiac types. Cardiac rhabdomyomas are considered as hamartomatous lesion because of their association with phacomatosis. Extracardiac type is further classified into adult, fetal and genital form, depending on the individual tumor's degree of differentiation. Adult head and neck rhabdomyomas are rare pathologies of adult patients, with a male predominance. The occurrence of multifocality is a rare manifestation of this uncommon lesion. Presenting symptoms are related to the location and dimension of the tumors and they include upper airway obstruction, Eustachian tube dysfunction, dysphagia and mucosal and neck mass. Because of their high rate of recurrence, radical resection is the treatment of choice of this kind of tumors. In this article is reported a rare and particularly large case of head and neck adult rhabdomyoma, presenting with an history of sleep apnea and night-time stridor.