Osteogenesis imperfecta (OI) or brittle bone disease is described as group of genetic diseases which cause bones to become fragile, thereby breaking easily. OI is rare, affecting around 6 to 7 per 100,000 of the population world over. However the impact on the quality of life can be debilitating. Primary cause of OI is attributed to gene mutations in COL1A1 and COL1A2. There two genes encode type I collagen types pro alpha-1 and 2. Bone matrix main component is type 1 collagen and any mutation in its genes can cause reduction in either structure or production of abnormal collagen. The defect in collagen leads to weakening of bones that will cause them fracture susceptible. We report the case of a neonate diagnosed with Osteogenesis Imperfecta (OI) Type II, presenting with multiple congenital fractures, respiratory distress, and other complications. This case highlights the challenges in the management of a neonate with OI, focusing on the immediate postnatal care, respiratory support, and the management of recurrent sepsis. The complexity of the condition was further compounded by associated anomalies such as inguino-scrotal hernia and transient metabolic disturbances. Despite intensive neonatal care, the