Giant congenital melanocytic nevi (CNM), present at birth and greater than 40 cm in size, exhibit an increased risk of malignant transformation and may be associated with neurocutaneous melanosis. We present the case of a twelve month old female who presented with a giant congenital melanocytic nevus involving the upper, middle, and a portion of the lower back with multiple satellite lesions on the face, trunk, and extremities. An MRI of the brain displayed areas of hyperintensity in the cerebral hemispheres, right thalamus, and right cerebellum, consistent with neurocutaneous melanosis, however the patient was asymptomatic. Awareness of the clinical features of giant CMN is important for early detection of neurocutaneous melanosis and appropriate treatment.
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