Infantile GM1 Gangliosidosis is a rare inherited metabolic disorder with an incidence of 1 in 1–2 lakh live births presenting with rapidly progressive neurological and systemic illness associated with skeletal abnormalities, facial dysmorphism , visceral organomegaly, ocular and cutaneous manifestations. We report a genetically proven case of an infant with skeletal, neurological, occular, visceral, cutaneous abnormalities