OBJECTIVE To highlight the advantages of the vertical midline incision in providing satisfactory cosmesis with complete excision of pediatric nasofrontal dermoid cysts. METHODS Retrospective case series of nasofrontal dermoid cysts in 4 patients treated at a single tertiary medical center from June 1, 2010, through July 31, 2012. The mean age at surgery was 2.5 years. The anatomical location of the nasofrontal dermoid cysts differed: (1) supratip extending through the upper lateral cartilages to the cartilaginous septum, (2) upper dorsum and subcutaneous tissue, (3) tip and supratip extending deep to the nasal bones with involvement of the anterior cranial fossae and dura, and (4) nasal tip extending deep to the level of the rhinion and involving the upper lateral cartilages and below the left medial canthus. Preoperative imaging was performed on all patients. There was one case of intracranial extension. All patients underwent surgical excision with the vertical midline incision. Nasal reconstruction was performed with local soft-tissue flaps (1 patient), regenerative tissue matrix (2 patients), and bone dust pate (1 patient). The patient with intracranial involvement also underwent a frontal craniotomy. All lesions were histologically confirmed as dermoid cysts. Mean follow-up was 1.5 years. There were no complications or recurrences. All patients had cosmetically acceptable scars. CONCLUSIONS A vertical midline incision with modifications to excise involved skin provides a satisfactory and cosmetically sound approach to congenital lesions of the nasofrontal region. It affords adequate exposure for complete excision and reconstruction. A multidisciplinary team consisting of a neurosurgeon, facial plastic surgeon, and pediatric otolaryngologist is needed to optimize outcomes.