BACKGROUND : We present the case of an adolescent girl with prominent clitoral swelling as the first symptom when she presented to the emergency department, and who was subsequently diagnosed with nephrotic syndrome. METHODS : A 14-year-old adolescent girl was admitted with painless clitoral swelling. She denied recent masturbation, itching, or discharge. She was within the last few days of menstruation. Physical examination revealed clitoral edema without erythema or genital edema. Urine dipstick test and microscopic evaluation revealed protein 2+, blood 3+, abundant erythrocytes and 9-10 leukocytes. A few days later, additional clinical findings, such as pretibial and facial edema, were diagnosed as nephrotic syndrome. CONCLUSIONS : This case is a reminder that clitoral swelling is to be considered a sign in the diagnosis of nephrotic syndrome, even when it occurs alone.