A case of Proteus syndrome in a 10 year old male is reported. Asymmetric progressive enlargement started on left side of face, trunk, left hand and left leg as he grew up, hypertrophied left eyelid, cataract changes in left eye, missing teeth on left maxilla and extra teeth on left mandible, depressed chest and red patches on left side of chest. Cardiomegaly, delayed milestones, slowed scholastic performance, sluggish speech were present. There are only few reports of such cases in adults are reported in medical journals and this syndrome has rarely reported from India.

Key Words: Hemihypertorphy, Proteus syndrome, Disproportionate and asymmetric overgrowth, diagnostic criteria; misdiagnosis; evaluation and management.