Only a few published case of fibrous hamartoma of infancy (FHI) have commented on the changes in the overlying skin. There are descriptions of individual cases with cutaneous hyperpigmentation, hypertrichosis and hyperhidrosis. It is interesting to describe our case because, to our knowledge, the altered pigmentation of the overlying skin, with hairs on the surface, and eccrine gland hyperplasia combined with abortive hair follicles has not been reported together in association with the FHI. We recommend that all the biopsies must be excisional including overlying skin, thus the epidermal and dermal adnexal changes can be assessed in the microscopic evaluation which could have relevant implications from clinical and embryological point of view.