Severe rat lung worm disease (RLWD) is an uncommon condition, but it can result in severe complications and can be difficult to diagnose, necessitating awareness on the part of physicians everywhere. We review the clinical manifestations and diagnostic dilemmas of severe RLWD based on a case in Hawaii. A 50-year-old man developed mild headache, a burning sensation in the limbs, fever, and strained urination nine days after consuming lettuce contaminated with parasitic nematodes (Angiostrongylus cantonensis [A. cantonensis]). In time, his headache became more severe, and he developed purple semi-circular stripes at the base of nail beds. He sought medical attention, but the diagnosis was delayed, likely due to unfamiliarity with the condition by the initial treating clinician. The diagnosis was eventually based on evidence of cerebrospinal fluid (CSF), eosinophils, and positive polymerase chain reaction (PCR) of CSF for A. cantonensis. Corticosteroid treatment was delayed, and albendazole was not administered due to a lack of availability. A greater awareness of RLWD on the part of physicians may have prevented these delays. [ABSTRACT FROM AUTHOR]