The genital skin may be affected by a variety of dermatoses, be it inflammatory, infectious, malignant, idiopathic or others. The Red Scrotum Syndrome is characterized by persistent erythema of the scrotum associated with a burning sensation, hyperalgesia, and itching. Its cause is unknown but proposed mechanisms include rebound vasodilation following prolonged topical corticosteroid use and localized erythromelalgia. It is a chronic condition and treatment is often difficult. In this paper, we review the etiology, the physical and histopathological findings, and the management of this condition. We also describe related conditions such as Red Scalp Syndrome, Red Ear Syndrome, and Red Vulva Syndrome. Finally, we summarize the different cases reported in the literature and discuss the features that help in the differentiation of red scrotum syndrome from its mimickers.