Lipoma of the clitoris: A differential diagnosis with in disorders of sex development

Introduction: Genital lipomas are a rare type of benign soft tissue tumors that may mimic a disorder of sex development (DSD). Genetic, chromosomal, hormonal and developmental alterations are differential diagnosis that must be taken into account. Consequently, medical staff should have wide knowledge of the possible differential diagnosis regarding DSD since this pathology constitutes a medical and social emergency. Results: A three-year-old female child with a congenital cutaneous superficial lipomatous nevus of the clitoris who arrived to our institution with a misdiagnosis of DSD since birth. Preoperative laboratory analysis, imaging studies and biopsy, lead to proper diagnosis and further surgical removal and clitoridoplasty. Conclusion: Differential diagnosis of DSD includes infrequent pathologies as the one described. Early approach of a multidisciplinary team is essential in the treatment of these patients.