Squamous cell carcinoma (SCC) of the skin usually occurs in older patients and commonly develops from actinic keratosis. Patients with xeroderma pigmentosum (XP) can acquire SCC at an early age. To our knowledge the youngest reported patient with XP and SCC was 8 years of age. We report a 19-month-old Lebanese girl with XP who presented clinically because of a rapidly growing mass of the medial part of her right lower lid that was biopsied and found to be squamous cell carcinoma. The mass was surgically excised with no evidence of recurrence after 2 years of follow-up. We describe our clinical experiences with this patient and have reviewed the available literature concerning XP and malignancy.