Perineuriomas are rare benign peripheral nerve sheath tumor composed exclusively of perineurial cells. They have been classified into two main types according to their location – intraneural and extraneural (soft-tissue). Extraneural perineurioma are uncommon, found mostly in the soft tissue of the extremities and trunk, rare at other site. They mainly affect adults and have been sometimes described in children but are extremely rare in infants. We present a case of extraneural (soft-tissue) perineurioma of scalp in a 3-month-old infant, highlighting clinical, pathologic, and immunohistochemical features along with a discussion of the main differential diagnosis of this tumor. This is also the youngest case ever reported in the literature.
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