Syringolymphoid hyperplasia with alopecia is an uncommon skin disorder described in 1969 by Sarkany. Since then only three additional cases have been reported, all in the European literature. We present a 59-year-old man with two persistent hyperpigmented hairless patches of 13 years duration. Biopsy specimens revealed characteristic hyperplastic changes of the eccrine glands and ducts and a dense lymphocytic infiltrate surrounding eccrine structures with "syringotropism". Perifollicular lymphocytic infiltration and changes consistent with follicular mucinosis and mycosis fungoides were also present. Imunophenotyping and gene rearrangement studies showed the cells to be primarily of the T helper phenotype with rearrangement of the surface receptor gene.