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Median cleft lip with nasal dermoid is a very rare occurrence. Midline nasal dermoid results from the abnormal fusion of embryological processes. They can present as asymptomatic midline nasal swelling, infection, or meningitis due to intracranial extension. Median cleft lip also results from failure of fusion of embryological processes and can be complete or incomplete or associated with other congenital anomalies or as a part of syndrome. We present a rare combination of both in a 7-month-old female child who was surgically treated.