The extracardiac juvenile rhabdomyoma is extremely rare in the field of Otorhinolaryngology. The tumour usually arises from the soft tissue of the face or from mucosal sites, especially the oropharynx and the oral cavity but only sporadic endolaryngeal cases have been described in literature so far with predominance of young males. Here, we describe the very rare case of endolaryngeal extracardiac juvenile rhabdomyoma in a 42-year-old male. Clinical examination showed a mass of the right vocal cord, resembling a cystic lesion. Microlaryngoscopy revealed a non-encapsulated lesion and histopathology including immunohistochemistry which consecutively led to the correct diagnosis. This case suggests that the endolaryngeal extracardiac juvenile rhabdomyoma can be easily confused with a vocal cord cyst. Malignant transformations have not been reported but recurrences have been described. When total excision cannot be accomplished, reoperation or narrow follow-up is indicated to prevent advanced revision surgeries.