True hermaphrodite is one of the rarest variety of Disorders of Sexual Differentiation (DSDs) and represents only 5% of cases of all. A 12 year-old presented with complains of pain and swelling right side lower abdomen with Right sided Undescended testis. On exploration Mullerian structures was present on right side and on left side testis was normal into left hemiscrotum. 

Keywords: True hermaphrodite; Persistent mullerian duct syndrome; Disorders of Sexual Differention.