We report on a patient presenting severe diarrhea, anemia, dystrophy, visceromegaly and hypertriglyceridemia, due to an extremely rare lysosomal storage disorder. CC, a male, third-born of consanguineous parents, during the neonatal period had a sepsis and diarrhea. He was referred to us at the age of 3-months for pale mottled skin, mild dehydration, severe dystrophy, chronic diarrhea, distended abdomen, hepatosplenomegaly, bronchial stenosis and mild axial hypotonia. Blood biochemistry showed: Hb 8.8 g/dl; triglycerides 173mg/dl. Fecal calprotectin (343 g/g) and steatocrit (>50%) were increased. Blood and urine cultures, serology for common pathogens, and sweat test were negative. Lymphocyte subpopulations and plasma immunoglobulinswere normal. Abdomenultrasound showedhepatosplenomegaly (with a non-homogeneous and hyperechogenic liver) and hyperecogenic adrenal glands. A diet with casein hydrolysateswas ineffective,while diarrhea and anemiaworsened. Screenings for metabolic disorders, including galactosemia, were negative. Based on the clinical features (anemia, visceromegaly steatorrhea and hypertriglyceridemia), and the presence of adrenal calcifications, Wolman’s disease (WD) was suspected. The diagnosis was confirmed by enzymatic assays of acid lipase in dried blood spots, and by molecular analysis of the LIPA gene. WD, with an estimated incidence of 1/500,000, is a rare lysosomal disease. Onset typically occurs in the first weeks of life with digestive disorders, failure to thrive, hepatosplenomegaly, jaundice. Thus, WD should be considered in the differential diagnosis of severe early-onset diarrhea, particularly because treatmentwith enzyme replacement is now available, and the prognosis is poor in untreated patients.