A XEN gel stent implant procedure was performed in a one-year-old child with severe unilateral congenital glaucoma. At the age of 6 weeks, an uncomplicated 360° trabeculotomy had been performed, which resulted in intraocular pressure (IOP) control for only 4 months. The gel stent implantation was performed ab interno without complications. However, 1 month later, the stent was repelled into the anterior chamber due to the elasticity of Tenon’s layer. A first revision surgery was performed, with excision of Tenon’s layer and implantation of a new gel stent under sight. At the age of 18 months, a second revision surgery was performed because of an encapsulated Tenon cyst with insufficient IOP control, again with the implantation of a new stent. At that time, a progressive upper eyelid swelling was apparent. Eyelid biopsy led to the diagnosis of neurofibromatosis type 1, presenting with an orbital plexiform neurofibroma. Further insufficient IOP control resulted in a cyclodestructive procedure and loss of light perception during follow-up. XEN gel stent implantation in congenital glaucoma in infants is more challenging than that in adult patients. Gel stent implantation ab interno may be difficult due to the thickness and elasticity of Tenon’s layer. Gel stent dislocation may occur, even months after surgery. Trabeculectomy might be a better approach after failed trabeculotomy in congenital glaucoma. An underlying systemic disease might become apparent late during follow-up.