Background: Magnetic resonance imaging (MRI) findings suggestive of neurocutaneous melanosis (NCM) have been reported in asymptomatic patients with giant congenital melanocytic nevi (GCMN). Objective: To investigate the presence of NCM and the clinical neurologic status of patients with GCMN involving the head and neck. Methods: Thirteen patients with GCMN involving the head and neck were clinically examined by pediatric specialists in dermatology, ophthalmology and neurology. Electroencephalograms, noncontrasted and contrasted computerized tomography (CT) scans and MRI were performed. Results: Eleven of 13 patients with GCMN of the head and neck previously considered asymptomatic were found to present mild but evident neurologic alterations. No signs of NCM were found in the CT scans or in the MRI. Conclusions: Patients with GCMN of the head and neck may have associated neurologic alterations not related to the presence of neurocutaneous melanosis.