Abstract: Mucinous eccrine nevus has been described as a variant of eccrine nevus. Only one instance of this variant, diagnosed in a 47-year-old woman, has been reported. We report a 2-year-old girl who had two 1 cm, asymptomatic, brownish nodules. Histologic analysis revealed a dermal lobulated proliferation of eccrine structures surrounded by a prominent mucinous stroma. This is thus, to the best of our knowledge, the first case of this extremely rare entity in a child.