Melanoma with prominent pigment synthesis or animal-type melanoma (ATM) is a very rare type of melanoma. Its histogenesis has not been elucidated and ultrastructural features have not been described in human beings. We present an additional case of ATM in a 28-year-old woman with positive sentinel node biopsy and provide the results of electron microscopic studies. Histopathologically, the skin lesion was composed of heavily pigmented neoplastic cells mostly arranged as large sheets, focally also in a nodular growth pattern. After bleaching, the neoplastic cells demonstrated round nuclei with 1 or rarely 2 conspicuous nucleoli and a prominent nuclear membrane and abundant, gray, slate-like cytoplasm. Some cells demonstrated round cytoplasmic inclusions. There was no nuclear pleomorphism, and only a few mitotic figures could be found after extensive search. Multiple areas of necrosis en masse of tumor cells were seen. The lymph node biopsy revealed a complete effacement of the lymph node architecture by the extensive proliferation of hyperpigmented cells in the parenchyma. Immunohistochemically, the same pattern of staining was seen on the bleached and unbleached slides both in the skin and in the lymph node. The neoplastic cells stained positively with MiTF (nuclei), NSE, NKI/C3, tyrosinase (weak), p53, and CD68. S-100 protein, HMB45, Melan A, Mac367, and lysozyme reacted negatively. Occasional cells (<1%) reacted with MIB-1. Ultra-structural studies revealed that the neoplastic cells possessed a large, indented nucleus with a prominent nuclear membrane, a single (para) centrally located nucleolus, and peripherally marginated chromatin. The cytoplasm was abundant and contained numerous single melanosomes and rare compound melanosomes. The melanosomes were in stages II to IV of maturation, with a marked predominance of stage II and stage III melanosomes. There was a high number of aberrant melanosomes with a wide variety of configurations. Melanophages were a minor component of the lesion. Our ultrastructural studies provide unequivocal evidence that ATM is a neoplasm of melanosome-producing cells. We also review the literature on ATM.