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This report explores the relationship between the autosomal recessive disorder Schwachman‐Diamond syndrome (SDS) and neurodevelopmental disorders such as attention deficit hyperactivity disorder (ADHD), autistic spectrum disorder (ASD), mixed receptive‐expressive language disorder and intellectual disability. It also reflects on the management of associated developmental, emotional and behavioural challenges. A six‐year‐old boy with SDS underwent comprehensive psychiatric and psychological assessment and was found to meet DSM‐IV‐TR (APA, 2000) diagnostic criteria for ADHD combined type and to have mild intellectual disability. A diagnosis of ASD was excluded. Management of his ADHD included psycho‐education, behaviour modification, educational recommendations and medication. This study adds to knowledge of Shwachman‐Diamond syndrome and management of co‐morbid neurodevelopmental disorders.