Although cerebral sinovenous thrombosis (CSVT) is an uncommon disorder in neonates, the incumbent morbidity, mortality, and adverse neurodevelopmental sequelae highlight the importance of establishing an early diagnosis with an appropriate therapeutic plan. The clinical signs and symptoms of the condition are subtle and invariably masquerade under the umbrella of a broad spectrum of neonatal illnesses. A high index of diagnostic suspicion is essential for investigating and initiating treatment in a timely fashion before major complications ensue. Recent advances in accessible radiographic techniques with reduced radiation exposure have facilitated rapid diagnosis of thrombosis in both the superficial and deep plexuses of the cerebral venous systems. The absence of large-scale randomized trials and solid prospective smaller-sample-sized studies of neonates with CSVT has compromised our ability to develop efficacious treatment decisions. In this review of the scientific literature we offer understanding of the complex etiology of CSVT and inherent problems involved in the diagnosis and treatment of the disorder and focus on the limitations in current follow-up. An approach to neonatal CSVT is proposed on the basis of the available evidence from guidelines, registries, prospective and retrospective infant studies, and case series.