Solitary necrotic nodule of the liver (SNNL) is a rare benign lesion with uncertain etiology characterized by a “completely necrotic core” and a hyalinized capsule containing elastin fibers (Journal of Clinical Pathology 36:1181–1183, 1983). We report herein a 26-year-old woman with a previ- ous diagnosis of rheumatoid arthritis, systemic lupus erythematosus, and Sjögren’s syndrome and no history of malignancy who presented with a complaint of diarrhea of 1-year duration. In the abdominal ultrasound, mul- tiple paraaortic, portocaval, and ileal lymphadenopathies (LAPs) have been found with the largest one being 2 cm in size. The biopsy of the iliac LAP showed reactive nodular hyperplasia. An abdominal CT disclosed an inci- dental hypoechoic, heterogenous mass sized 27 × 27 mm close to segment VI of the liver. A trucut biopsy of this lesion was made, and clinicopatho- logic features of the specimen were compatible with a solitary necrotic nod- ule of the liver. Here, we discuss the diagnosis and the clinical course of this rare entity in light of current literature.