Pigmented Spitz nevus is a benign melanocyte skin lesion with distinct clinical features and it is frequently found on the extremities. However, it rarely occurs on acral area of the body, and such a case has not yet been fully documented. We present a case of acral pigmented Spitz nevus occurring on the foot, and this mimicked acral lentiginous malignant melanoma. Clinicians should be well aware of this entity and its possible clinical presentations. Since acral pigmented Spitz nevus is benign in nature, making the correct diagnosis is important to avoid unnecessary mutilating or excessive surgery.
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