We herein report a case of Spitz nevus which occurred in a 6-year-old girl with acute lymphocytic leukemia. The lesion was a well delimited, symmetric, 1.0 * 1.0cm sized, dome-shaped light pinkish nodule with a shiny surface on her forehead. The histopathological findings of the excisional biopsy specimen revealed mild epidermal hyperplasia ; numerous uniform nests of spindle cells and epitheloid cells with sparce pigmentation along the dermoepidermal junction; colloid body-like eosinophilic globules ; multinucleated giant nevus cells in the epidermis and edema in the upper dermis. Since the complete excision of her lesion was accomplished, there have been no new lesions over the past year. (Korean J Dermatol 1998;36(3) - 487-492)