RATIONALE: To report the management of recalcitrant neurotrophic keratopathy in a pediatric patient affected by pontine tegmental cap dysplasia (PTCD) using topical human recombinant nerve growth factor (hrNGF, Cenegermin 20 μg/ml). To the best of our knowledge the present case is one of the few described in patients with congenital NK treated with Cenegermin, and the first in a patient affected by PTCD. PATIENT CONCERNS: A 9-year-old patient, affected by PTCD with bilateral cranial nerve V1 and VIII palsies, was referred to our hospital for visual disturbances and redness of the right eye due to persistent neurotrophic epithelial defect. The patient presented marked developmental delay, ataxia, bilateral hypoacusia, and bilateral corneal severe hypoaesthesia. Ocular history revealed multiple treatments in order to treat neurotrophic ulcer in the left eye. Four years later, he developed a persistent epithelial defect with corneal anesthesia in the right eye. DIAGNOSES: The impaired trigeminal nerve function, due to the underlying congenital disease, led to the development of moderate NK (stage II) in the right eye and a mild NK (stage I) in the left eye. INTERVENTIONS: Cenegermin 20 μg/ml eye-drop was administered in both eyes. Treatment was continued for 8 weeks. The patient was assessed after 4 and 8 week of treatment. At each follow-up visit, treatment efficacy and adverse events were evaluated. OUTCOMES: The use of Cenegermin eye drops facilitated the remarkable resolution of the neurotrophic keratopathy and the improvement of corneal sensitivity in both eyes. No local or systemic adverse events were observed. LESSONS: Topical Cenegermin 20 μg/ml was well-tolerated and may represent a valuable therapeutic option in the management of pediatric neurotrophic keratopathy.