Background <p>Posterior Polar Cataract Combined with a cyst in the posterior segment of the vitreous has been described in one previous case report in 1946. Otherwise, there is no literature on this potential association. We describe a case of posterior polar cataract (PPC) combined with vitreous cysts.</p> Case presentation <p>A 26-year-old man complained of shadowed vision in his right eye (20/20) since childhood. Slit lamp examination revealed a ring-shaped opaque plaque in the superior central part of the posterior capsule, attached to a pigmented vitreous cyst. AS-OCT demonstrated thin-walled and intact cystic structures behind and adjacent to the posterior lens capsule.</p> Conclusions <p>The coexistence of PPC and vitreous cysts is a rare entity that requires careful evaluation and management. The presence of pigmentation and hemorrhages on the vitreous cyst seems to support the hypothesis that the vitreous cysts may be a cystic choristoma from the primitive hyaloid system.</p>