In 1910, Darier and Civatte described in details an unusual melanocytic tumor characterized by rapid growth on the nose of a young child. They could not state whether the tumor was benign or malignant. In 1947, Sophie Spitz described the same lesion as juvenile melanoma in which prognosis was frequently excellent. Later, the study was revised and it was concluded that juvenile melanoma was a benign tumor and can affect adults. Although, the prognosis was mostly excellent, Spitz reported in one of 13 cases fatal metastases from nevus Spitz. In 1999, Barnhill et al described one fatal case of the patient for whom it was thought to have typical Spitz nevus. Nowadays, there is still a lack of consensus about histopathology and also a terminology of the tumors that are neither typical nevus Spitz, neither malignant melanoma. All histopathological, clinical and ancillary criteria must be weighed in the final interpretation of epitheloid/spindle cell lesion. At the present, the final diagnosis remains pathohistological, with important emphasis given to clinical impression. Persistently changing lesion indicates malignancy potential of the lesion. Barnhill recommends that all Spitz tumors are completely excised. Atypical tumors should be excised with wider margins up to 1 cm. Patient should be carefully monitored by regular examinations for recurrence and metastasis.