A lateral cervical cyst occurred in a 30-year-old man with hemophilia A was reported. The cyst was located in the anterior portion of the sternocleidmastoid muscle, and was adjacent to the parotid gland. Amylase, GOT, GPT, γ-GTP and LDH activities of cyst contents were higher than those in patient's serum samples. Especially the amylase activity was extremely high. Histopathologically, the lining of the cyst wall was consisted of columnar or cuboidal cells. In addition serous gland and lymphocyte accumulation without germinal center were observed in some places of the connective tissue. The electron microscopic view had a strong resemblance to that of Varthin tumor. It is strongly suggested that our case was a parotid retention cyst.