Proliferating pilomatricoma is a proliferative variant of pilomatricoma. Although it is considered as a benign tumor, local recurrence may occur. We report a case of a 49-year-old Japanese man with 3-year history of an asymptomatic subcutaneous tumor in the nuchal area. Histological evaluation demonstrated a cystic lesion lined by a basaloid epithelium at the periphery and filled with eosinophilic cornified material containing shadow cells in upper part of the tumor, and multilobular proliferation of basaloid cells in association with small foci of shadow cells in the remaining part. Based on these findings, the diagnosis of proliferating pilomatricoma was made. No recurrence has been observed during a 3-year follow-up.