There has been only one documented case in the English literature with the diagnosis of primary Squamous Cell Carcinoma (SCC) of the nipple; we present a further case of a primary SCC of the nipple, thus raising awareness to the skin or breast specialist of this possible presentation for SCC. We present the case of a 34 year old lady who presented to our plastic surgery unit with an erythematous, scaly lesion on her right Nipple Areola Complex (NAC). The lesion was histologically confirmed on biopsy to be an SCC and subsequently formally excised. Histology confirmed complete excision of the lesion with adequate margins with no lymphovascular or perineural invasion. This case report describes a rare presentation of a primary moderately differentiated SCC of the nipple. Although SCC of the nipple is a rare diagnosis, in view of its similar presentation to Paget disease of the nipple, it must be considered and careful examination of the histology must be performed in order to ascertain a definitive diagnosis. Patients presenting with lesions of the NAC cannot be assumed to have either Paget's disease or SCC and biopsy should be performed before arranging further investigations or treatment, as the pathways for the two conditions can be very different.