Tufted angioma, also known as angioblastoma, is a rare benign vascular neoplasm of the skin and subcutaneous tissue. The natural history of tufted angioma is slow, progressive growth for several years, and tends to remain stable thereafter, resulting in disfiguring appearance. The main choice of treatment for tufted angioma is complete surgical excision, but local recurrence is frequent. Here, we report a 33-year-old woman with a four-year history of a steadily increasing pink to violaceous plaque on her right arm accompanied by spontaneous, progressive pain over the last seven months. Histology revealed multiple separated lobules of spindle cells throughout the dermis and subcutaneous tissue to the deep margin, with numerous mitoses but no cellular atypia, the small capillary-sized vessels scattered within the lobules, which were consistent with tufted angioma. To our satisfaction, her pain disappeared and the whole plaque was significantly improved after a four-month course of external application with imiquimod 5% cream.