Rosai-Dorfman Disease is a rare disorder. We report a case of ten-month Saudi male infant who presented with persistent painless left neck mass for the previous three months, accompanied by fever, odynophagia, night sweats and weight loss. Physical examination showed enlarged left upper cervical lymph node measuring up to five to seven cm and filling up the gap anterior to sternocleidomastoid muscle and angle of mandible. It was cystic, fluctuant, semi-mobile, non tender. Computed tomography (CT) neck with IV contrast were performed, which revealed non enhancing hypodense lymph node at upper cervical region. Lymph node was surgically removed. Histopathology confirmed Rosia-Dorfman Disease. The patient made good recovery and was discharged on oral prednisolone 10mg daily for one month. A one -year follow-up did not reveal any recurrences.