Three cases of primary cutaneous neuroendocrine carcinoma involving the face are reported. In the first patient the tumor developed in the scar of a previously excised basal cell carcinoma of the nose, recurred locally, and metastasized to a regional lymph node 2 ½ years after initial treatment. In the second and third patients the tumors were located on the forehead and were removed with a wide excision. In the second patient the tumor metastasized to regional lymph nodes 1 month after treatment. Light microscopic examination in all patients showed an undifferentiated, small cell, dermal tumor with prominent epidermotropism, forming Pautrier-like microabscesses. Immunohistochemical staining for neuron-specific enolase was positive, and ultrastructural examination revealed numerous cytoplasmic neurosecretory granules and paranuclear aggregates of intermediate-sized filaments. The literature concerning epidermal involvement by primary neuroendocrine carcinoma of the skin is reviewed; the relation with basal cell carcinoma and treatment are discussed.