Introduction.Chromobacterium violaceum is an opportunistic human pathogen, associated with significant mortality, and has been reported in patients with chronic granulomatous disease (CGD), a genetic condition causing impaired phagocytosis. Case presentation. A 28-year-old man with a history of CGD presented with fever, pharyngitis, cervical lymphadenopathy and internal jugular vein thrombosis, following travel to the rural Solomon Islands. C. violaceum was recovered from his blood. The patient recovered after treatment with meropenem and trimethoprim/sulfamethoxazole. Conclusion. To the best of our knowledge, this is the first case report of internal jugular vein thrombophlebitis (Lemierre's syndrome) caused by C. violaceum in a patient with CGD. A review of the literature demonstrated that the diagnosis of C. violaceum preceded the diagnosis of CGD in the majority of cases. This case emphasizes the importance of this organism in patients with CGD who live in or visit tropical areas.