Kimura disease (KD) is a rare benign chronic inflammatory condition of unknown cause, usually affecting young men of the Asian race. It is frequently associated with nephrotic syndrome. In this report, we present an uncommon case of KD in a 40-year-old Saudi man with sickle cell disease who presented with swelling on the right side of his face. CT scan of the head and neck showed the asymmetrical appearance of both parotid glands: the right side appeared heterogeneously enlarged, with adjacent moderate-to-significant fat stranding. Histologically, hyperplastic changes in lymphoid tissue were observed. The patient underwent superficial parotidectomy and was then followed up till the healing of the surgical site with no complications.