Alexander disease is a rare autosomal recessive disease, characterized by degeneration of white matter of the central nervous system. It is a leukodystrophy that usually occurs in early childhood and is characterized by megalocephaly, demyelination and multiple Rosenthal fibres. Specific magnetic resonance imaging findings and gene mapping are essential to diagnose the disorder. The patient mostly presents with frontal predominance; megalocephaly, seizures, spasticity, psychomotor deterioration, excessive vomiting, difficulty in swallowing and speaking, poor coordination, and loss of motor control, slowly progressive paresis, bulbar signs, brisk reflexes, but often with an intact mental state and are may often be fatal. This case report deals with the dental Management of a 10years old child brought to the out-patient department of paediatric and preventive dentistry, Guru Nanak Institute of Dental Sciences and Research, with a chief complain of pain and blackish discoloration in his lower front teeth region and frequent bleeding from gums, suffering from Alexander disease.