BACKGROUND: Deep penetrating nevus (DPN) is a distinct variant of melanocytic nevus and remains a histopathologic challenge to pathologists because of its resemblance to blue nevus, malignant melanoma, pigmented Spitz nevus, and congenital melanocytic nevus. It often goes unrecognized due to its relative rarity. CASE PRESENTATION: Here we report a case of DPN of the left anterior leg in a 51-year old female. A brief review of the literature shows that these lesions have a distinct growth pattern and cellular morphology that can differentiate these lesions from other entities including malignant melanoma. CONCLUSION: It is important to recognize these features because DPN carries a better prognosis than malignant melanoma.