A 17-year-old girl born to nonconsanguinous parents presented a history of optic atrophy, Retinitis pigmentosa for eight years, followed by squint and nystagmus after two years. She developed progressive bilateral sensory neural hearing loss at the age of 12. She had menarche spontaneously at 14 years, but her cycles have been irregular ever since. She was diagnosed with dilated cardiomyopathy at 16 years, followed by Type 2 diabetes at 17 yrs. Hence the clinical diagnosis of Alstrom syndrome (AS) was made based on clinical criteria. She also developed renal impairment and needed frequent hospital admissions with congestive heart failure. She died at the age of 21 years due to cardiac failure. Interestingly she was not obese and insulinresistant as reported earlier but lean and insulinopenic. The rarity and insulinopenia, an unusual aspect of the case, deserved to be published.