Mastocytosis is a heterogeneous disease of bone marrow origin, characterized by local or diffuse increased growth and accumulation of clonal mast cells in the skin and/or in internal organs. The skin is the organ most frequently involved, but others may be affected as well. The purpose of this article is to present the oral management of a child with urticaria pigmentosa/maculopapulous cutaneous mastocytosis requiring dental treatment under general anesthesia. The multidisciplinary team approach involving the relevant medical specialists is discussed to emphasize the significance of coordinated patient management.