An 11-day-old boy was found to have a lesion of the left lower eyelid and medial canthus. The mass was present at birth and subsequently enlarged, and was subtotally excised when the child was 2 months old. Histopathologic examination disclosed a fascicular to slightly nodular pattern of predominantly spindle-shaped cells. Special stains demonstrated fibroblastic and smooth-muscle features in individual cells, and infantile myofibromatosis was diagnosed. Sixteen months postoperatively, no evidence of recurrence was noted. We believe this is the first case of infantile myofibromatosis of the solitary type involving the ocular adnexa of a neonate to be reported in the English literature. Infantile myofibromatosis of the solitary type appears to have an excellent prognosis and may be treated with conservative resection if the tumor infiltrates important adnexal structures.