Objective: Atypical Ewing sarcoma/primitive neuroectodermal tumor with the melanocytic differentiation is a very rare, malignant tumor which occurs in infants and adolescents. It is localized mainly in soft tissues. Its biological behavior is aggressive, but its response to the chemotherapy is prognostically good. Method: A 4-month old male is presented with the 17×11 cm tumor of the soft tissue, localized in the right front over the cranial margin of the orbit. The tumor was surgically exstirpated and then sent to the histological investigation with the additional immunohistochemistry and the FISH. Results: Microscopically, the tumor is hypercelullar and consisted of oval to polygonal cells with the vesicular nuclei, the prominent nuclei and the amphophilic cytoplasm. In other parts of the tumor, there are smaller neoplastic cells with hyperchromatic nuclei. Some neoplastic cells contain the melanin pigment in the cytoplasm, which was positive in Fontana-Masson method. The immunohistochemical stains for vimentin, AE1/3, CK7, CD99, HMB-45, FLI-1 were positive. The FISH investigation demonstrated the translocations (t 11;22, q24;q12) in 95 % of neoplastic cells and (t 21;22, q12;12) of 5 % ones.