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We present a case of acquired unilateral agminated angiofibromas in a 26-year-old patient with onset at the age of 18. He developed over 100 pigmented dome-shaped papules on the left side of his nose, cheek and chin. Histological examination showed acanthosis and dermal capillary proliferations, fibrosis and lymphohistiocytic infiltrates. Staining with CD-31 showed increased vascular formation in the dermis. Staining for S-100 protein, HMB-45, NKIC-3 and Masson-Fontana staining showed no nevocytes or melanocytes in the dermal tissue, suggesting that the lesions are a segmental expression of tuberous sclerosis. However, multiple agminated angiofibroma without a relationship to tuberous sclerosis or a nevocytic or melanocytic formation cannot be ruled out for certain.