The Spitz nevus is an uncommon melanocytic nevus. These lesions classically appear in childhood as a red, dome-shaped papule. They appear rarely in adults and may be pigmented. The Spitz nevus can develop suddenly and grow rapidly, reaching a 1-cm diameter in 6 months or less. There are 3 classes of spitzoid neoplasms: typical Spitz nevus, atypical Spitz nevus, and spitzoid melanoma. The diagnosis should be cautiously differentiated, especially in children. Immunohistochemistry and molecular studies have been helpful in differentiating difficult cases; however, no set of criteria has been accepted to predict biological behavior of atypical Spitz nevi.