Spitz nevi are benign melanocytic proliferations that are characterized by spindled and/or epithelioid nevomelanocytes. It shows wide array of clinical appearances such as pyogenic granuloma, intradermal nevus, hemangioma, or melanoma. Herein, we report a case of Spitz nevus with very peculiar dermoscopic and clinicohistopathologic findings. A 15-month-old boy presented with an erythematous nodule on the left upper arm. The lesion occurred only 1 month ago and the size of the lesion increased rapidly. No subjective symptom such as itching or pain was noted. There was no trauma or medication for nevus lesion on his past history. Dermoscopic findings showed numerous dotted vessels and whitish lines. Histopathologic findings revealed aggregation of epitheloid and spindled nevus cells in dilated lymphatic vessels. There have been several reports of intralymphatic nevus cell aggregates (ILNA). It can occur with various types of nevi including intradermal nevus, compound nevus, and Spitz nevus. A pathomechanism of ILNA is suggested by some authors as a "benign metastasis" that is caused by mechanical forces, such as local minor traumas. However, there was no trauma history in our patient. We discuss a dermoscopic and clinicohistopathologic finding of ILNA occurring in a Spitz nevus, which has never been reported in a Korean dermatologic literature.