A 12-year old Japanese girl presented with a 10-year history of keratosis lichenoides chronica on the extremities and oral mucosa. She had a poor response to topical corticosteroids, maxacalcitrol and 20% urea ointment. Phototherapy and systemic immunosuppression were refused by the parents. She was then treated with topical tacrolimus ointment 0.1% and 20% urea ointment under occlusive dressings. Within 2 months the lesions and pruritus had dramatically decreased. No adverse effects were seen. Thus topical tacrolimus ointment is worthy of consideration in refractory keratosis lichenoides chronica (Oyama, N. et al. Eur J Dermatol 2011, Advance publication).