A 13-month-old girl, born of non-consanguineous marriage, presented with complaints of pearl like lesion on right nipple since birth. The lesion had slowly increased to size of a pea, but was not associated with any pain or itching. Growth and development were normal. Examination showed 5×5 mm pearly white nodule involving right nipple, with normal surrounding skin (Fig. 1). There was no erythema, induration or tenderness around the lesion. Left nipple was normal. A diagnosis of congenital milium of nipple was made, and parents were counselled about the benign and self-limiting nature of the lesion.

Fig. 1 Pearly white nodule involving right nipple, with normal surrounding skin.

Milia (singular, milium) are inclusion cysts in the epidermis. The lesion develops due to entrapment of keratin. It appears as yellow to pearly white papule on the skin. It can be primary that arise spontaneously or secondary resulting from trauma such as sunburns or bullous skin disorder. Primary congenital milia are common in neonate and may be seen in almost 40% of full-term neonate. They generally appear over the cheeks, nose or eyelids. Solitary and large milium is seen over areola, scrotum or vulva. Solitary milium should be differentiated from the other lesions like syringoma, calcinosis cutis, trichoepithelioma and fibroma. Milium of the nipple should also be differentiated from witch’s milk seen in some neonates due to maternal hormones, and galactorrhea due to excessive secretion of prolactin. Congenital milia resolve spontaneously but rarely may persist for a long duration. Excision of the lesion in the nipple area should be avoided as it can lead to functional and aesthetic complications, especially in a female child.