AIM: To evaluate the ophthalmic and anesthesiologic management of cataract surgery in children with Lowe syndrome receiving lens removal, the development and management of secondary glaucoma. METHODS: This retrospective case series included 12 eyes of 6 children with genetically verified Lowe syndrome receiving cataract removal. Information regarding the type and duration of surgery and total anesthesia time were recorded. Additionally, intra- and postoperative complications were noted as well as clinical examinations such as visual acuity and funduscopy. RESULTS: All children received simultaneous bilateral cataract surgery at the mean age of 8.98±3.58wk. Lensectomy combined with posterior capsulotomy and anterior vitrectomy was performed in all children. The mean time for cataract surgery per eye was 35.83±8.86min, whereas the total time of surgery was 153.33±22.11min. The mean extubation time and duration at recovery room was 42.33±22.60min and 130.00±64.37min, respectively. During surgery, a decrease of oxygen saturation below 93% was found in only one child. During the postoperative follow-up, nystagmus (6 children) and strabismus (5 children) was commonly found in contrast to no case of visual axis opacification. Secondary glaucoma developed in five eyes of three children, which was treated with topical eye drops in only one child. A trabeculectomy was performed in both eyes of one child, whereas removal of syechia and an iridectomy in one eye of one child. CONCLUSION: Bilateral simultaneous cataract surgery under general anesthesia is a safe surgical procedure in Lowe syndrome children. The glaucoma screening with intraocular pressure measurements is crucial in the postoperative management of Lowe syndrome patients to avoid additional visual impairment.