Acquired progressive lymphangioma (APL) is a rare, benign proliferation of lymphatic capillary origin, which is characterized histologically by dermal vascular channels and a 'dissection of collagen' appearance. We describe a 30-year-old patient with an extensive, refractory APL on the right breast, which slowly developed over a period of 23 years. Pathologists and dermatologists should be aware of this entity, as early surgical treatment may be totally curative when the lesion is limited in size.