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A 12-year-old girl presented to Manipal Teaching Hospital with quadriparesis of 8 months’ duration. Examination revealed a hyperpigmented patch over the chest wall with overlying hypertrichosis, musculoskeletal anomalies, upper limb asymmetry and ipsilateral breast hypoplasia. MRI scan revealed cranio-vertebral junction anomaly and spina bifida occulta at the cervical spine level. Histopathological examination of the skin revealed findings consistent with Becker's nevus. Based on the patient's clinical presentation and investigations, a diagnosis of Becker's nevus syndrome was made. However, she was managed conservatively as surgical intervention was not suitable in her case. The authors review Becker's nevus syndrome and its clinical manifestations below.