Desmoplastic Spitz nevus (DSN) is an uncommon variant of melanocytic nevus rarely encountered in dermatological practice. Herein, we describe a 54-year-old male who presented himself with a cutaneous tumor arising from the left arm. Histology revealed an intradermal proliferation of somewhat pleomorphic, epithelioid, spindled melanocytes in a background of desmoplastic stroma. A perineural invasion of tumor cells was found. Proliferative and mitotic rates were minimal. The tumor was diffusely positive for S-100 protein, PNL-2, and SOX-10, and only occasionally reactive for melan-A and HMB-45. The final diagnosis of DSN was established. Although DSN is a completely benign tumor, it may result in diagnostic pitfalls. Due to its unusual histopathological features, it may be confused with a malignant desmoplastic melanoma. A knowledge of the clinicopathological differences between the two prognostically distinct skin tumor entities is essential for a differential diagnosis.