Introduction and importance: Dandy-Walker malformation is a posterior cranial fossa anomaly, characterized by the absence or hypoplasia of the vermis and cystic dilatation of the fourth ventricle, resulting in elevation of the tentorium and torcula. This condition can present with a broad range of neurological and developmental symptoms, highlighting the importance of early recognition and intervention to improve patient outcomes.

Case presentation: This case describes a 15-month-old male presenting with head enlargement and motor dysfunction. A computerized tomography scan identified absence of the cerebellar vermis and ventriculomegaly. The patient underwent ventriculoperitoneal shunt placement, leading to significant improvement (head size, motor function) and complete recovery from convulsion over a six-month follow-up.

Clinical discussion: The clinical presentation primarily originates from cerebellar dysfunction, impacting balance, coordination, vision, motor skills, cognition, and behavior. This condition occurs sporadically and is frequently associated with hydrocephalus. Treatment is centered on managing symptoms and related comorbidities.

Conclusion: Dandy-Walker malformation is a congenital anomaly of the posterior fossa that causes a wide range of neurological and developmental challenges, primarily hydrocephalus. Effective management requires a multidisciplinary team, including pediatric surgeons and pediatricians, with timely consultation with a pediatric neurosurgeon and neurologist being essential.

Keywords: Dandy walker; Head enlargement; Hydrocephalus; Motor; Posterior fossa; Vermis.

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