Mucinous eccrine naevus (MEN) is an extremely rare variant of eccrine naevus. There are only nine reported cases in the English literature. We report a 3-month-old boy with a congenital lesion on the lower back, and review the other cases in the literature. In most cases, MEN presents before puberty. The commonest presentation is a unilateral, solitary, brownish nodule without hyperhidrosis occurring on the legs. The diagnosis of this condition is confirmed by the characteristic histopathological features. Treatment options may be considered if symptomatic.