Systemic corticosteroids have long been the mainstay of treatment for pemphigus patients. However, the necessity of their high-dose and long-term administration has brought about a number of complications, sometimes causing significant morbidities. Maintaining a balance between therapeutic and undesirable effects of medications is not always easily achievable. Therefore, additional treatment modalities are frequently needed to control side-effects. Kaposi's sarcoma (KS) is a rare, potentially life-threatening complication in this setting. Due to the rarity of data on pemphigus-associated KS treatment, the best therapeutic approach is still undecided. Here, we report two cases of pemphigus patients who had developed extensive KS as a result of severe immunosuppressive therapy and were successfully treated with paclitaxel. In addition, we performed a review of literature to assess the results of the previously employed treatment modalities in this setting.