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Linear and whorled nevoid hypermelanosis (LWNH) is an extremely rare pigmentary mosaicism. It is characterized by hypermelanic macules that follow a Blaschko’s lines pattern. The disease has been found to be associated with the presence of trisomy 4, 7, 13, 14, 18, 20 and chromosome X-linked disorders involving mutations at the Xp21-p22 locus. However, LWNH usually presents as mosaicism. Only 16 to 31% of the cases are associated with neurological and cardiac disorders. Here is reported the case of a 6-year-old female patient with a history of generalized absence seizures and sleep disorders, that were associated with LWNH.