Spasmus nutans is classically described as a triad of nystagmus, head nodding and torticollis. It occurs in children, beginning in infancy and usually disappears in childhood. It is uncommon but its prompt recognition by ophthalmologists, paediatricians and neurologists can provide reassurance to the family of its benign nature. It is important to distinguish it from more serious conditions like gliomas which may present with signs mimicking spasmus nutans. We describe a case of a 3-year-old boy with acquired horizontal pendular nystagmus associated with head nodding and torticollis. The diagnosis of spasmus nutans, its implications and its management are discussed.