Tuberous sclerosis complex (TSC) is an autosumal dominant progressing disease, causing the development of benign tumors in all organs and tissues of human body. Astrocytic hamartomas are the characteristic ocular manifestation of TSC. Rare iris abnormalities have been reported in patients with TSC and include focal areas of stromal depigmentation and atypical coloboma and hamartomas of the IPE and ciliary epithelium in histopathologic findings in two enucleated cases.We report a case of TSC with iris hyperpigmentation as a presenting sign.

A 39-year-old man presented to us with complaint of different iris color of both eyes. Visual acuity was 7/10 in the right eye and 10/10 in the left eye .On examination, the right eye had hyperpigmentation of iris and fundus with multiple lesions suggestive of retinal astrocytic hamartomas.

Spectral domain OCT demonstrated elevated lesions with high reflectivity arising from inner retinal layers and causing posterior shadowing. On systemic examination, he had multiple small scattered angiofibromas over the cheeks and nose and shagreen patches. Brain CT scan showed multiple subendymal calcific nodule.

Diagnosis of TSC is not difficult if a physician is familiar with clinical presentation of the disease