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Verrucous epidermal nevus is a common type of keratinocyte hamartoma present at birth or occurring later in life. Widespread multiple epidermal nevi (EN) may reflect genetic or chromosomal mosaicism. Blaschko has documented characteristic linear, zosteriform, unilateral or systematized patterns with streaks and swirls. They are resistant to treatment with the risk of recurrence. Giant congenital nevus unius lateralis (NUL) is known for its benign course with good supportive treatment however one particular case had a fatal neonatal outcome due to associated complications. Two interesting rare cases will be reviewed here.