Abstract
INTRODUCTION AND IMPORTANCE: Lipoma is a benign mesenchymal tumour especially noted in adults, adolescents including older age groups. Generally, 1 to 2 % of the population is affected by lipomas, including superficial to deeper tissues as well as the organs. But however, lipomas are rarely seen at birth more so ever in the perineal or perianal region. Such a rare case of congenital perianal lipoma having associated external genital anomaly has been reported here, in view of its rarity and in its management. CASE PRESENTATION: Author reports here a rare case of congenital perianal swelling in a neonate, its associated anomaly and its management. CLINICAL DISCUSSION: Congenital perianal lipoma is rare especially in neonates; affecting both genders equally. CONCLUSION: Complete evaluation for other associated anomalies along with surgical excision of perianal swelling is mandatory for their ultimate pathological diagnosis and for future management in a neonate.