Megaprepuce (MP) is a rare and challenging condition characterised by an excessive inner prepuce, paucity of penile skin and an extremely narrow phimotic ring. The aetiological factors leading to its development are poorly understood. A variety of surgical techniques have been described in the last 26 years mostly with small number of patients and short follow-up. It is also highly likely that some series have in the past included different variants of inconspicuous penis combining concealed penis, MP and webbed penis. This article is a systematic review of the literature on Megaprepuce; in particular the embryology, history, aetiology, and the surgical techniques available for the correction of this unique penile anomaly will be presented and discussed in this study.