These are case series with six patients who presented unusual neck swelling. Worldwide, the most common swelling in the neck region is a enlargement of cervical lymph nodes. Each country has a different etiology and geographical distribution for cervical lymphadenopathy. Tuberculosis, metastatic cancers, and lymphomas are the commonest causes. However, in clinical practice, we encounter rare neck swelling. Such swellings are due to their less common and unusual presentation, and its clinical diagnosis is rarely attained. In such cases, proper diagnosis and management are required to attain the desired results. Materials and methods: In our tertiary healthcare center, we encountered such case in ENT OPD. Five cases were evaluated in this article along with their management. Such rare causes of neck swellings are therapeutically challenging, as they cause diagnostic dilemmas. Results: Histopathology and immunohistochemistry were the mainstay of diagnosis, and surgery was curative in most cases.