It's unusual for systemic lupus erythematosus (SLE) to present with diffuse alveolar hemorrhage (DAH) as the earliest presentation. Diffuse alveolar hemorrhage (DAH) refers to the effusion of blood into the alveoli due to damaged pulmonary microvasculature. It’s a rare but severe complication of systemic lupus associated with a high mortality rate. It occurs in three different overlapping phenotypes, which are acute capillaritis, bland pulmonary hemorrhage, and diffuse alveolar damage. The illness develops over hours to multiple days. Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with many organ involvements with high morbidity and mortality percentage. Central and Peripheral nervous system complications generally develop during the course of the illness and actually uncommonly from the beginning of the illness. Guillain- Barré syndrome (GBS) is a rare autoimmune polyneuropathy usually occurring post-viral, post vaccination, or surgery. Systemic lupus erythematosus (SLE) has been associated with several neuropsychiatric manifestations and development of GBS. GBS as the first presentation of SLE is exceedingly rare. Here, we present the case of a patient with diffuse alveolar hemorrhage and Guillain-Barré syndrome as an atypical presentation of systemic lupus erythematosus (SLE) flare.