A 43-year-old Caucasian female, with known 46XY chromosome pure gonadal agenesis and systemic hypertension, presented with upper motor neuron weakness of the right face and upper limb. She was also noted to have livedo reticularis and advanced finger clubbing. Left-sided cerebral infarction was confirmed on brain computed tomography (CT) and, following further investigations, a diagnosis of Sneddon’s s syndrome (SS) was made. Three years later, she went on to develop adenocarcinoma of the right lung, which sadly claimed her life within 4 months. To our knowledge, this is the first reported case of SS in association with finger clubbing and pure gonadal agenesis.