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Introduction: median raphe cysts (MRC) are uncommon lesions of the male genitalia. They can be found anywhere from the distal penis to the perianal area. They are caused by an incomplete closure of the median raphe during embryologic development. Objective: to present a case of MRC in an infant and provide a review on diagnosis, treatment and follow-up. Case report: one year and three months old boy, with seven 0.5 cm sized cystic lesions along the scrotum median raphe, noticed since birth. A soft tissue ultrasound demonstrated a well-circumscribed hypoechogenic solid image without any evidence of vascularity at Doppler ultrasound. Surgical excision was conducted and histopathology confirmed the diagnosis of a median raphe cyst of the perineum, showing good progression in follow-up. Discussion: 75% of cases of MRC are asymptomatic, thus their diagnosis is often delayed. Not knowing this condition leads to confuse MRC with other similar cysts or tumors. The diagnosis is mostly clinical, although ultrasound may help to exclude other anomalies such as vascular anomalies. Surgical excision is the treatment of choice to prevent superinfection or local discomfort due to its location and size. Conclusion: Recognition of this uncommon pathology is imperative. A comprehensive evaluation of the pediatric patient is recommended to achieve an adequate diagnosis and treatment and provide appropriate information to the parents.
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