<b><i>Introduction:</i></b> Spitz nevus is an uncommon, benign melanocytic proliferation that primarily appears on face, trunk or lower extremities of children. This lesion may share clinical and microscopical characteristics with melanoma, making it a diagnostic and management challenge. <b><i>Case Report:</i></b> A 13-year old male presented with an asymptomatic chronic dermatosis located on the third left-hand nail. Cutaneous examination revealed a homogeneous dark brown melanonychia which extended up to the cuticle. Upon dermoscopy, longitudinal bands measuring less than 3 mm wide of heterogeneous colors ranging from light to dark brown, and positive Hutchinson’s sign were observed. <b><i>Discussion/Conclusion:</i></b> We report the second case of a Spitz nevus ungually localized which strongly resembled an ungual melanoma with a positive Hutchinson’s sign upon dermoscopy. Describing the infrequent presentation and location of the Spitz nevus poses an opportunity to establish diagnostic and management criteria in the near future.