We report a case of a 10-year-old boy with Laugier-Hunziker-Baran syndrome. He had pigmented, oval or round macules with well defined borders on the lip as well as the tip of his finger. These pigmented macules were first noticed 8 years previously. On examination, no intestinal polyposis or adrenal dysfunction suggestive of Peutz Jeghers syndrome or Addison’s disease was noted. A biopsy of a pigmented macule from the lip revealed melanin pigmentation of the basal layer with occasional melanophages in the upper dermis. In Japan, 53 cases of Laugier-Hunziker-Baran syndrome have been reported. We reviewed the complications and the treatment and discussed the difference between Laugier-Hunziker-Baran syndrome and Peutz-Jeghers syndrome from the point of view of the literature and our case.