Graham Little-Piccardi-Lassueur syndrome (GLPLS) is a rare lichenoid dermatosis defined by scarring alopecia, loss of pubic and axillary hairs and progressive development of horny follicular papules variously located. Topical or systemic corticosteroids, retinoids or PUVA therapy are the treatments usually proposed and these have partial and temporary benefits. We describe the effectiveness of cyclosporin A in a case of GLPLS at the dosage of 4 mg/kg/day. At the end of treatment, substantial reduction of both perifollicular erythema and follicular hyperkeratotic papules was observed. After 3 months of follow-up, besides the results already obtained, a few areas of hair regrowth in the scarring patches and a more consistent improvement of the follicular papules were detected. We believe that cyclosporin A could be effective mainly in the initial phases of this rare variant of lichen planopilaris, before the development of severe follicle damage, either by interfering with the acute inflammatory processes or by limiting the progression of the disease. To the best of our knowledge, this is the first report showing a good and persistent therapeutic effect of cyclosporin A in GLPLS.