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Steroid treatment introduced for pediatric nephrotic syndrome has multiple side effects, one of which is glaucoma. Although steroid glaucoma is often improved only by eye drops, there are a few cases that require glaucoma surgery. We experienced a case with severely increased intraocular pressure found precociously after start of steroid treatment at the onset of nephrotic syndrome. Although severe steroid glaucoma was successfully treated by trabeculotomy in both eyes, increased intraocular pressure recurred after steroid treatment for the relapse of nephrotic syndrome. This case carried angle dysgenesis, generally shown in pediatric glaucoma, in addition to the general steroid responder component. Therefore, this case might have a rapid increase in intraocular pressure after steroid treatment, in which glaucoma surgery was urgently required. Because steroid glaucoma can follow rapid and severe course as our case, intraocular pressure should be checked and controlled immediately as possible.