Congenital milia of the nipple are extremely rare, and standard treatment has not yet been established because nipple preserving excision is problematic due to the location. Although most reports show excisional biopsy resulting in good outcomes without recurrence, there is a lack of consensus about treatment modality, with several studies suggesting that incisional evacuation by needle, or a ‘wait and see’ approach represent sufficient treatment. This case report is about a recurrent case after incisional evacuation for congenital milium of the nipple. We recommend nipple preserving excision with exfoliation of the milial capsule as being the most appropriate treatment modality for congenital milium of the nipple.