Microspherophakia (MSP) is a rare congenital zonular dysplasia characterized by increased anteroposterior lens thickness and reduced equatorial diameter, resembling a spherical shape. The related ocular manifestations of MSP include shallow anterior chamber, lens derived high myopia, ectopia lentis and secondary glaucoma. In the early stage of MSP, vision acuity may be improved by glasses. Cataract surgery is necessary once secondary lens dislocation and glaucoma occur. A 72-year-old male hospitalized patient was reported who complained increased blurred vision of his right eye for 2 years. In the past 2 years, the power of his binocular glasses was increased to -10 diopters without significant improvement in visual acuity. Lens dislocation and phakic insufficiency caused by MSP was diagnosed after he attending clinics at the Eye and ENT Hospital of Fudan University, Shanghai, China. Phacoemulsification with scleral sutured intraocular lens (IOL) implantation surgery and anterior vitrectomy were performed. One month after operation, macular edema was found at first follow-up. Compound pingdingmu granule was taken orally and Bromfenac sodium eye drops were applied three times a day. Two weeks later, visual acuity of his right eye was improved significantly and macular edema was eliminated dramatically.