Basal cell nevus syndrome (BCNS) is a rare autosomal dominant multisystem disorder due to a mutation in the PTCH1 gene. Dermatologic manifestations include basal cell carcinoma (BCC) and palmoplantar pits. This case of childhood-onset BCC arising in a child of Filipino descent with BCNS illustrates the importance of early detection and diagnosis, which will then facilitate early counseling and management of this lifelong condition. An 11-year-old Filipino-American girl presented with multiple variegated pearly papules on the face, clavicular area and upper back. She also had multiple palmoplantar pits. Shave biopsy of a papule showed BCC. Trans-abdominal ultrasound revealed bilateral ovarian calcifications. Jaw and chest radiography were unremarkable. Skin lesions were initially treated with ablative erbium:YAG laser and later recurred after 3 months. Imiquimod cream was then started which resulted in partial resolution of lesions after 12 weeks. The dermatologist's role in the diagnosis of BCNS is integral, especially in this case where the patient is a seemingly well child with predominantly dermatologic manifestations. Clinical findings of BCNS, including BCC, are recurrent. Lifetime monitoring, counseling and adequate patient education and appropriate treatment of lesions are central to the management of this condition.