Abstract
The perineal groove is a rare congenital malformation that mainly affects females. Stephens and Smith first described it in 1968 as a moist groove between the vulvar fork and the anus without alteration of the vestibule, urethral or vaginal introitus, and hypertrophy of the labia minora bordering the groove posteriorly to join or surround the anus. Perineal groove is usually asymptomatic and with spontaneous resolution. Due to its lack of knowledge and scarce documentation in the literature, its diagnosis is often erroneous and patients and their families are subjected to stressful events, unnecessary studies and treatments. A literature review is conducted to increase awareness of the existence, treatment, and prognosis of this medical condition.