Background: Incontinentia Pigmenti (IP), also known as Bloch-Sulzberger disease is a rare X linked dominant Genodermatosis with skin lesions as a typical feature, accompanied by multisystem developmental disorders that can involve teeth, eyes, the central nervous system and other organ systems. The incidence is 1.2/100,000 births. The most characteristic manifestation is cutaneous, which occurs along Blaschko’s lines. 

Clinical description: Here we report case of a six-year-old developmentally normal girl child admitted to paediatric department with poor scholastic performance, hyperpigmented lesions on skin, abnormal dentition, and poor eyesight. On examination child was found to have typical dermal, dental findings of Incontinentia Pigmenti along with loss of vision and squint in right eye which on further evaluation comes out to be persistent hyperplastic primary vitreous and vitreous haemorrhage. 

Management: Child was managed conservatively along with ophthalmic and dermatologic evaluation. MRI brain revealed right optic nerve atrophy and persistent hyperplastic primary vitreous with white matter demyelination changes. She was referred to vitreoretinal surgeon for surgical management. 

Conclusion: Ophthalmic associations include retinal neovascularisation, microphthalmia, strabismus, optic nerve atrophy, cataract and retrolental mass. Although ocular manifestations are not the most common, they are often highly debilitating and can lead to blindness if not identified early. 

Sapna Gupta, MD (Pediatrics) with a Fellowship in Pediatric Neurology, is an Associate Professor at MLB Medical College, Jhansi. She has been working in the field of pediatric neurology for more than 17 years, serving the people of the Bundelkhand region. With over 20 years of undergraduate and postgraduate teaching experience, she has played a significant role in medical education and mentorship.She serves as a thesis guide for postgraduate students and has contributed to academic assessments as an external examiner for undergraduate, postgraduate, and pediatric neurology fellowship programs at various medical colleges across India. She has several research publications in reputed journals and is the North Zone Editor of the Journal of the Academy of Pediatric Neurology of India (JAOPN). In addition to her academic and clinical work, she is actively involved in various academic activities and has served as an Organising Secretary for national pediatric neurology conferences.