Five cases of a distinctive variant of desmoplastic Spitz nevus are reported. To the best of our knowledge, this tumor has never been described previously. Clinically, it presents itself as a solitary papule on the extremities of young adults. Microscopically, it shows predominance of solitary melanocytes with epithelioid appearance over cell nests. They are embedded in a prominent fibrous stroma with many densely arranged, small blood vessels with plump endothelia not seen in other Spitz nevi. Because of its resemblance to a vascular tumor, the name angiomatoid Spitz nevus is proposed for this lesion. Absence of recurrences or metastases after complete excision in all cases supports the benign nature of the tumor.