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Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare cause of cervical lymphadenopathy. It is a potentially life-threatening hypersensitivity reaction, commonly characterized by fever, rash, hematological abnormalities, and multi-organ involvement. Its association with agranulocytosis is even rarer, with fewer than 10 cases describing the coexistence of DRESS with agranulocytosis reported in the English literature. An otherwise well 40-year-old female presented with a sore throat and cervical lymphadenopathy, with investigations revealing DRESS and agranulocytosis secondary to carbamazepine. DRESS and agranulocytosis are serious, potentially life-threatening adverse drug reactions which can initially present as cervical lymphadenopathy. As carbamazepine is considered first-line therapy for certain chronic neuropathic conditions such as trigeminal neuralgia, clinicians should be aware of the varying clinical presentations of both conditions.