Syringolymphoid hyperplasia with alopecia is an uncommon, but histopathologically distinct, skin disorder that has been reported to occur with and possibly represent a syringotropic variant of cutaneous T-cell lymphoma. We report 2 patients with syringolymphoid hyperplasia with alopecia. Both had CD4-positive infiltrates; 1 also demonstrated loss of CD7. One patient had evidence of T-cell clonality by gene rearrangement studies, but neither had histologic evidence of cutaneous T-cell lymphoma. Because the natural progression of syringolymphoid hyperplasia with alopecia remains to be fully explained, close follow-up of patients is advised.