Polythelia is a congenital anomaly in which there is a third or more nipples. Although it is a relatively rare condition, it is frequently associated with other congenital malformations including the cardiovascular, urogenital, and skeletal systems. It may occur sporadically or with a familial tendency with some genetic factors. We report an association of polythelia with congenital hypogonadotropic hypogonadism (CHH) in a young male. The patient was complaining of delayed puberty. He was short in stature and had hypogonadism. On physical examination, a third nipple was found below the normally located nipple along the left mammary line. The case was carefully evaluated to exclude other disorders. It has been suggested that every newborn can be checked for the presence of accessory nipples which could indicate another internal problem in its body.