Neck node enlargement is a common clinical presentation in pediatric age group. Here we are presenting a rare cause of neck node enlargement in a pediatric patient, called Kimura’s disease (KD). KD is a rare chronic inflammatory disease with angiolympahtic proliferation. The etiology of KD is still unknown in medical literature. Painless unilateral soft tissue mass in the head and neck region is the commonest clinical presentation. This disease should be suspected when there are painless neck nodes, hypereosinophilia and hyper IgE particularly in male Asian patient. The diagnosis of KD is confirmed by histopathological examination. There are three main treatment options in KD like surgical excision, corticosteroids and radiotherapy. Clinicians need to be aware of KD and help to limit the number of laboratory tests and reduce the possibility of malignant lesions.