Objective: Eosinophilic fasciitis (EF) is an uncommon rheumatological disorder that may complicate with aplastic anemia (AA). We aimed at reviewing the literature on AA associated with EF.

Materials and Methods: Published articles were selected using the following entry terms: "eosinophilic fasciitis", “Shulman’s disease” and “aplastic anemia”. All-important data were collected.

​Results: Case descriptions on 16 patients were reported. Age varied from 18 to 62 years, most males and AA appeared, in general, within the first year after EF diagnosis. In three patients, rituximab was used, and bone marrow transplantation was done in 4 of them. Paroxysmal nocturnal hemoglobinuria (PNH) clone was searched in 6 of the reviewed patients and was positive in 2.

Conclusions: AA in EF showed a predominance of older males; most of them developed AA within 12 months after EF diagnosis and treatment included glucocorticoid, ATG and immune suppressive drugs, bone marrow transplantation and rituximab.