Kimura disease (KD) is a rare chronic granulomatous inflammation disease with unknown causes, which is common in young Asian males. Local lymph node drainages are characteristic of the disease, the most common of which occur in the salivary glands. Other reported drainages occur in the axilla, popliteal fossa, groin, and upper extremities. Unfortunately, there not many characteristic manifestations that typically present in patients with KD. Sometimes only increased peripheral blood eosinophils and serum IgE concentration are the only positive presentations. Therefore, it is very difficult for physicians to diagnose KD. We report herein on two cases of KD. Both of them presented in the soft tissues of the upper extremities. Special attention was given to the MRI findings of the two patients with a view to disclosing specific characteristics that should help in the realization of clinical diagnosis.