Nevus lipomatosus superficialis is a hamartoma characterized by ectopic mature adipose tissue in the papillary dermis. The condition was first described by Hoffman and Zurhelle in 1921. Two types of nevus lipomatosus superficialis are recognized, namely, the classical multiple type (also known as the Hoffman-Zurhelle type) and the solitary type. A 6-year-old girl presented with skin-colored, 1.7x1.3cm sized, well-defined, dome-shaped nodule on the left palm for several years. Histologic findings showed mature adipocytes between collagen fibers in both the papillary and deep dermis. These findings were consistent with the nevus lipomatosus superficialis. The large lesion was totally excised by fillet flap. At follow-up, the excision region healed with a cosmetically acceptable appearance. Although the solitary type of nevus can develop anywhere on the body, the occurrence on the hand has never been reported. Herein, we report the first case of a child with nevus lipomatosus superficialis on the left palm with fillet flap.