Background: Spherophakia (congenital bilateral anomaly in which the lens of the eye is small, spherical, and prone to subluxation) is a rare entity associated with systemic disorders including Weill-Marchesani syndrome, Marfan’s syndrome, Alport’s syndrome, Klinefelter’s syndrome and hyperlysinaemia. One of its well known complications is angle closure glaucoma (increased intraocular pressure secondary to physically obstructed anterior chamber angle). It is debatable whether prophylactic treatment for spherophakia is necessary or if treatment should only be performed in symptomatic patients.
Case Report: We describe an 18-month-old child with bilateral spherophakia complicated by an acute on chronic angle closure glaucoma in the right eye. To our knowledge, this is the youngest child to be reported with pupillary block secondary to anterior lens dislocation in spherophakia.
Conclusions: It seems that early intervention with lensectomy and anterior vitrectomy allows for adequate control of the intraocular pressures in patients with spherophakia.

Keywords: lensectomy, Glaucoma, spherophakia